Myocardial accumulation of metaiodobenzylguanidine (123I-MIBG) is low [H/M = early: 1.72.


Delayed: 1.34 ]. The circled areas indicate heart.


On March 25, 2019, abdominal CT showed a mass (4.2*3.5cm) in the tail of pancreas, and multiple liver nodules were observed (A, B).


Abdominal enhanced CT showed multiple liver nodules (C, May 2019).


On October 10, 2019, the patient underwent transcatheter arterial chemoembolization with local anesthesia (D).


Positive expression of CgA.


Medical image.


Glucagon. In primary tumor tissue.


The Ki-67 index was 5~10.


25~30%. In the primary tumor and liver metastases, respectively.


Non contrast axial CT initial scan in the emergency room demonstrated no significant intracranial pathology. However the soft tissue windows demonstrated loss of fat planes in the right parapharyngeal space (blue arrow) and masticator space (red arrow). (For interpretation of the references to colour in this Figure legend, the reader is referred to the web version of this article).


Axial STIR MRI sequence demonstrates hyperintensity (oedema) in multiple neck spaces including masticator space adjacent to the lateral pterygoid muscle (blue arrow). There is mucosal congestion within the nasopharynx (red arrow) with high signal noted posteriorly in the longus capitis muscle (green arrow) suggestive of extension through the retropharyngeal space. Note fluid secretions within the right mastoid air cells (yellow arrow). (For interpretation of the references to colour in this Figure legend, the reader is referred to the web version of this article).


Axial FDG PET-CT demonstrates mild increased avidity in the right deep neck spaces including carotid and masticator spaces (arrow). The "low grade" nature of the tracer uptake suggests infective cause rather than malignant.


(a) CT scan showing swellings in the lymph nodes (arrows).


(b) Low-density areas in the spleen were also observed (arrows), suggesting metastasis from a malignant tumor of the left ovary.


18FDG avidity was observed in the lymph nodes (arrows).


In the spleen (arrows).


(a) Microscopic findings of the resected ovarian tumor and lymph nodes. Atypical cells with clear cytoplasm grew papillary, tubulocystic, and focally solid pattern (hematoxylin and eosin [HE.


(b) Non-caseating epithelioid granulomas were observed in the pelvic lymph node as well as in the spleen where there were no metastatic lesions (HE).


Preoperative image showing nasomaxillary mass causing cheilo-nasal dysmorphism.


(a) Preoperative magnetic resonance imaging showing soft-tissue mass in the nasomaxillary region.


(b) Delivery of the excised mass.


(c) Closure of the defect.


Photomicrograph showing cellular spindle cell lesion arranged in fascicles with "herringbone pattern".


Photomicrograph showing vimentin positivity in neoplastic cells.


Eighteen months' postoperative follow-up showing complete regain of facial architecture.


Gross necropsy: lung tumours in an Asiatic lion with haemangiosarcoma.


Gross necropsy: liver tumours in an Asiatic lion with haemangiosarcoma.


Preoperative X-ray.


Preoperative CT-scan.


Postoperative X-ray.


Nodular lesions on the right leg.


Acid-fast Bacilli in large numbers, grouped together in cigar-bundle appearance. Stained with modified Ziehl-Neelsen staining, x100.


Dermis showing diffuse polymorphous inflammatory infiltrate with foamy histiocytes, neutrophils, plasma cells, and lymphocytes; stained with H and E, x100.


Fite-Faraco stain demonstrating acid-fast Bacilli, x100.


Imaging of patient with germline BAP1 mutation. A. Retinal examination revealed a melanocytic tumor with irregular margins involving the majority of the retina.


Imaging of patient with germline BAP1 mutation. B. CT of the abdomen prior to surgery identified a 32 mm lobulated lesion in segment 4B of the liver as indicated by the arrow.


Pedigree of the patient's family with a BAP1 germline mutation. Black Squares indicate a diagnosis of malignant mesothelioma. Arrow indicates the presence of BAP1 mutation as determined by sequencing studies. Two of the patient's children passed away with renal cell carcinoma (RCC) and acute lymphocytic leukaemia (ALL).


Intense scaling and edema over the patient's face.


Angio-CT scan. (a) Cavernous segment of the right internal carotid artery, below the line of projection of the planum sphenoidale (red dotted line).


Angio-CT scan. (b) Cavernous segment of the left internal carotid artery with its posterior genu above the line of projection of the planum sphenoidale (red dotted line).


Angio-CT scan. (c) Enlargement of the sella turcica and left carotid artery laterally displaced.


Angio-CT scan. (d) Superior displacement of the cavernous segment of the internal carotid artery and an aneurysm close to the communicating segment of the internal carotid artery (white arrow).


Intraoperative image showing the carotid aneurysm (white arrow), compressing the left oculomotor nerve.


Schematic representation of the aneurysm (white arrow), compressing the oculomotor nerve.


Image after microsurgical clipping (black arrow). AC: Anterior clinoid; CN II: Optic cranial nerve; CN III: Oculomotor cranial nerve; FL: Frontal lobe; ICA: Internal carotid artery; R: Fixed retractor.


Postoperative left carotid angiography. Frontal.


Oblique.


Lateral) showing the clip adjacent to the cavernous segment of the left internal carotid artery, with complete aneurysm occlusion.


Sella turcica magnetic resonance imaging consistent with pituitary macroadenoma with apoplexia. Axial fluid-attenuated inversion recovery.


Coronal T1 with gadolinium.


Sagittal T1 with gadolinium).


A panoramic view of the jaws shows a well-defined
unilocular radiolucency with central foci of calcification displacing
the roots of the maxillary canine and premolar.


Medical image.


Thick walled encapsulated lesion with intraluminal
epithelial proliferation and extracapsular fibrous tissue with irregular
mineralization (H&E- whole slide digital scan) Multinodular
intraluminal solid and cribriform proliferation, CEOT-like areas
and extracapsular fibrous lesion with irregular mineralization (H&E
x 40).


(A). Intraluminal nodules consisting of cuboidal or columnar epithelial cells in the shape of nests, rosette-like patterns and duct- like
spaces with interlacing strands of oval, angular and elongated epithelial cells between nodules. Double layered basaloid epithelial cells in trabecular
or cribriform configurations between and connecting cell rich nodules.


(H & E x 200) (B). Tumor droplets (Eosinophilic amorphous
material) between the cells and in Rosette-like structures (H&E x 400).


CEOT -like area. (A). Island of polygonal epithelial cells with intracytoplasmic homogenous material and calcification (H& E x
400).


CEOT -like area. (B). Polyhedral epithelial cells exhibiting intercellular bridges and calcification (H&E x 600).


Congo-red positive islands of amorphous eosinophilic (amyloid like) material (x 200).


Congo-red positive islands of amorphous eosinophilic (amyloid like) material (x 200). . Light microscopy.


Congo-red positive islands of amorphous eosinophilic (amyloid like) material (x 200). Apple green birefringence
- partially crossed polarz, (C). Crossed polarz (x200).


(A). Fibrocellular tissue interspersed with irregular trabeculae of bone and cementum-like tissue and foci of hemorrhage (H&E x 40).


(B). Fibrocellular connective tissue interspersed with ginger root-like trabeculae showing few lacunae and ovoid cementicles with prominent
resting lines (H& E x 100).


Confluence of woven and lamellar bone in maturing trabecule, (A). H&E x 200.


(B). Polarized light microscopy x 200.


Right groin showing multiple excavating ulcers, left groin shows 'Groove sign of Greenblatt' and multiple tense bullae with erosions over thighs and penile shaft.


Photograph of sectioned gross specimen shows a dark red to brown soft tissue tumor which composed of two ovoid mass.


Photomicrograph demonstrates a columnar cells surrounding vascular cores. (H & E, x 200).


MR image taken 2 years after operation shows no local recurrence.


Patient diagnosed as esthesioneuroblastoma presenting with proptosis and diminished vision in right eye.


Coronal cuts of CECT showing homogeneously enhancing soft tissue involving right side of sinuses and orbit.


Axial cut of CECT of the paranasal sinuses (PNS) showing hyperintense mass involving right side of nose and orbit.


Midsagittal view of a T2-weighted.


A contrast-enhanced T1-weighted. Magnetic resonance imaging (MRI) of the lumbar spine demonstrating an intradural extramedullary spinal lesion (white arrowhead) from L1 to L2 with avid contrast enhancement.


(c) Axial view of a contrast-enhanced T1-weighted MRI of the lumbar spine demonstrating high-grade compression of conus medullaris and cauda equina from an intradural extramedullary lesion (white arrowhead).


Postoperative magnetic resonance imaging, sagittal.


Axial. Views, confirmed complete tumor removal.


Histologic findings of the lesion. (a and b) Show spindle cells, collagen fibers, microcystic changes, and hemosiderin deposits on Hematoxylin Eosin staining (black circle and arrowhead).


Histologic findings of the lesion. (c) Shows a Ki67 <1.


Histologic findings of the lesion. (d) shows uniform S-100 protein immunoreactivity.


Patient with cutaneous neurofibroma.


Cutaneous neurofibroma over the trunk.


Patient's father with cutaneous neurofibroma.


Gingival neurofibroma.


A) OCT of the left eye at presentation reveals subretinal fluid overlying the macular lesion.


B) OCT image three weeks after presentation and before treatment demonstrates complete anatomical resolution.


The main clinical manifestation of the patient before surgery. (A) shows the obvious cataract in the right eye of the patient.


The main clinical manifestation of the patient before surgery. (B) shows the exudative retinal detachment in the inferior part of the retina, accompanied by naevus-like multifocal reddish patches in the superior part of the fundus.


The main clinical manifestation of the patient before surgery. Total retinal detachment was observed in the posterior pole of the retina when the patient was in the supine position (C).


The main clinical manifestation of the patient before surgery. (D-F) show the similar clinical manifestation of the left eye.


Pathological examination of biopsies collected from the choroid during surgery. (A) shows hematoxylin-eosin (HE) staining of the biopsies, which revealed a predominant spindle-shaped melanocytic cell mass.


Pathological examination of biopsies collected from the choroid during surgery. Immunohistochemical staining showed that the tissue was positive for antibodies against S-100.


Pathological examination of biopsies collected from the choroid during surgery. And negative for antibodies against Ki67.


Postcontrast sagittal magnetic resonance image showing a heterogeneously enhancing tumor involving the lateral, third and fourth ventricles. Tumor is noted to extend into the cistrna magna (arrow).


Postcontrast coronal magnetic resonance image demonstrates tumor extension into both lateral ventricles. It extends through the third ventricle into the fourth ventricle.


Axial MR images of a cystic lesion on the anterior portion of the spinal chord which demonstrates isolucency on T1-weighted MR images.


Hyperintensity following gadolinium contrast on T2 weighted image.


MR imaging revealing a non-enhancing cystic-appearing lesion growing off the conus medullaris at L1- L2 in the spinal with hyperintensity on a sagittal T1-weighted image.


Hypointensity on a T2-weighted image.


Microscopy images of the endodermal cyst revealing a simple ciliated columnar epithelium at 10x.


Medical image.


40x. Magnification.


Ulcerated basal cell carcinoma on the arm.


Several foci of basal cell carcinoma within the epidermal nevus on the back.


Close-up view.


(a) Fundus photo of the LE of case 1, showing the optic nerve head granuloma and serous retinal detachment.


(b) Ultrasonography of the LE of case 1, showing significantly raised lesion over the optic disc.


(c) FFA of the LE of case 1, showing severe leakage of dye at the optic disc along with microaneurysms.


(a) Fundus photo montage of RE of case 2, depicting granuloma at the superior margin of the optic disc, serous retinal detachment of the macula, and retinal phlebitis with "candle wax drippings" seen superiorly.


(b) Fundus photo montage of the RE of case 2, at 3 months of follow-up. Granuloma has resolved and cuffing has been replaced with sheathing. Hard exudates can be seen in the macula after resolution of the subretinal fluid.


H&E-stained section at 10x magnification shows a poorly differentiated ductal carcinoma (left side of field) with areas of chondroid (cartilaginous) differentiation (right side of field).


H&E-stained section at 20x magnification shows malignant neoplastic cells within chondroid matrix material.


Chest radiograph showing left lung upper lobe mass.


Perioperative magnetic resonance imaging. The tumor showed isointensity on T1-weighted imaging.


Perioperative magnetic resonance imaging. And slightly hyperintensity on T2-weighted imaging.


Perioperative magnetic resonance imaging. After administration of contrast medium, the lesion was heterogeneously enhanced (C).


Perioperative magnetic resonance imaging. Postoperative spinal MRI confirmed a subtotal resection of the tumor. Coronal.


Perioperative magnetic resonance imaging. Sagittal.


Perioperative magnetic resonance imaging. Axial.


Images of a dog with CRGV (Case 1) illustrating how a dark-red necrotic, cutaneous lesion in the umbilical region expanded in size over the course of approximately 48 h (a-c). Credit: Gregory Cameron MVB, AllPets Drogheda.


Images of a dog with CRGV (Case 2) illustrating an extensive, erythematous necrotic lesion involving the skin of the caudoventral abdomen and medial aspect of left hindlimb.


In the surgical specimen, there were irregular nests of polygonal cells with heteromorphic nuclei. Some tumor cells had foamy cytoplasm, and sebaceous differentiation was observed (hematoxylin-eosin x20).


A: Chest computed tomography images show multiple lung metastases.


B: After two cycles of chemotherapy with carboplatin and paclitaxel, all lung metastatic nodules regressed.


Non-invasive diagnostic workup including ECG, echocardiography, DE-CMR, and CT.


Figure showcasing the timeline of events.


Invasive diagnostic workup including surgery and histopathology, confirming the diagnosis angiosarcoma of the heart.


(a) Preoperative view of Case 2. A 1.0 cm x 1.5 cm ulcer was noted in the perineal lesion (arrow).


(b) The ulcer was excised completely, and a bilobed perforator flap was designed.


(c) Intraoperative view of flap transfer. The large flap was transposed to cover the perineal defect, and the small flap was transposed to cover the donor site of the large flap.


(d) Three-month postoperative image showing a favorable appearance.


(a) Postoperative X-ray cervical spine, anterior-posterior view.


(b) Lateral view, due to very short neck, lower screws are not visible.


Preoperative magnetic resonance images (MRI). Preoperative MRI showed right frontal mass lesion. The lesion was isointense in T1-weighted MRI.


Preoperative magnetic resonance images (MRI). Preoperative MRI showed right frontal mass lesion. Hyperintense in T2-weighted MRI.


Preoperative magnetic resonance images (MRI). Preoperative MRI showed right frontal mass lesion. Hyperintense in fluid attenuation inversion recovery image.


Preoperative magnetic resonance images (MRI). Preoperative MRI showed right frontal mass lesion. Homogenous enhanced with gadolinium intravenous injection. Axial image.


Preoperative magnetic resonance images (MRI). Preoperative MRI showed right frontal mass lesion. . Coronal image.


Preoperative magnetic resonance images (MRI). Preoperative MRI showed right frontal mass lesion. . Sagittal image).


Intraoperative findings and postoperative MRI findings. During surgery, tumoral invasion beyond the dura mater was observed (a). When the dura was opened, a well-demarcated, firm, spherical tumor was observed beneath the arachnoid membrane.


Intraoperative findings and postoperative MRI findings. The tumor was elastic, hard, grayish in color, and loosely adherent to but separable from the dura mater (b).


Intraoperative findings and postoperative MRI findings. The tumor was totally removed piece by piece, with the adjacent brain tissue and dura (c).


Intraoperative findings and postoperative MRI findings. Postoperative MRI showed the total removal of the tumor. Axial view.


Intraoperative findings and postoperative MRI findings. . Coronal view.


Intraoperative findings and postoperative MRI findings. . Sagittal view).


Chest x-ray (October 2011).


Gluteus mass biopsy histopathology examination (December 2011).


Left frontal brain tumor histopathology examination (December 2011).


Staging CT showing bilateral renal masses, pancreatic cysts and multiple lung metastases.


Hyperkeratotic plaques on trunk and limbs and crusted lesions on her scalp and external ears.


Hyperkeratotic plaques on trunk and limbs and crusted lesions on her scalp and external ears (A, B, C, D).


Microscopical examination of scrapings from the hyperkeratotic lesions from trunk showed (arrow) mites (M) and eggs (E) (magnification x40).


Magnetic resonance imaging L spine. Sagittal slices demonstrates lesion on T2.


Magnetic resonance imaging L spine. , T1.


Magnetic resonance imaging L spine.T1 with contrast.


Magnetic resonance imaging C spine. Sagittal slices demonstrates lesion on T2.


Magnetic resonance imaging C spine. , T1.


Magnetic resonance imaging C spine.T1 with contrast.


Preoperative frontal view of the patient showing multiple swellings on the anterior scalp.


Preoperative superior view of the patient showing multiple swellings on the anterior scalp.


Intraoperative view after surgical excision of multiple swellings.


Excised specimen showing multiple swellings with a glistening, smooth, faintly lobulated surface. Note the cut section of the tumor showing solid mass inside.


Histopathology showing lobules of epithelial cells arranged in a jigsaw or mosaic pattern. Prominent red basement membrane-like structure encircles the tumor lobules. Each lobule shows a peripheral lining by dark basaloid cells and an inner larger and paler zone of cells which was suggestive of dermal cylindroma.


Postoperative frontal view of the patient after one week.


99mTc-methylene diphosphonate bone scintigraphy showing diffusely increased left rib cage uptake (anterior a, posterior b; arrows). Transaxial single photon emission computed tomography (SPECT) and SPECT/CT images (c, e; arrows) show diffusely increased radiotracer uptake involving the left-sided ribs.


99mTc-methylene diphosphonate bone scintigraphy showing diffusely increased left rib cage uptake (anterior a, posterior b; arrows). Transaxial CT (bone window, d) shows normal left-sided ribs with regular cortical outlines.


99mTc-methylene diphosphonate bone scintigraphy showing diffusely increased left rib cage uptake (anterior a, posterior b; arrows). Transaxial CT (soft tissue window,. Shows a pleural-based soft tissue density lesion in the left lung lower lobe. Arrow head) with diffuse circumferential pleural thickening. Arrow).


Ultrasound abdomen demonstrates hyperreflective pancreas, indistinguishable from normal retroperitoneal fat.


Contrastenhanced axial CT images demonstrate atrophic pancreas with total fat replacement. The entire pancreas has low attenuation value (HU = -120).


Main pancreatic duct appears to be stenotic (rather than dilated), suggesting chronic severe obstruction;. Noncontrast axial CT image shows solitary calculus in distal pancreatic duct near the ampulla of Vater.


Contrastenhanced axial CT image demonstrates mildly dilated small bowel loops with wall thickening and dilution of contrast medium.


Large radiolucent area extending until the lower margin of the mandible on the right (white arrows). Vertical bone resorption toward the proximal root of 46 (black arrow).


Area of a great accumulation of foamy histiocytes.


Spindle cells and many foamy histiocytes.


Spindle cells surrounding fragments of bone trabeculae.


Orthopantomography 6 months postoperatively. The defect at the area of the surgical site as well as the post-extraction socket of 46 are distinguished. Nothing remarkable concerning the bone healing process is observed.


Macroscopic view of the 2.0-cm subcutaneous tumor on the right elbow.


A-c Cell morphology of the primary and recurrent tumors. A; The primary tumor showed irregularly arranged collagen fibers mixed with scattered proliferating fibroblast.


B; The first recurrent tumor, composed of slimmer spindle cells.


C; The second recurrent tumor, composed of slimmer spindle cells. Cells in the recurrent tumors were more monomorphic and showed higher cellularity.


D-f Immunostaining for Ki-67. The Ki-67 labeling index of the primary tumor was 2% ,that of the first.


Second recurrent tumors 10.


7% , respectively.


Depicts the macules and areas of hyperpigmentation along with severe ocular abnormalities.


Reveals the perioral scarring due to actinic chilitis.


Intraoral view revealed dark brown and gray pigmentation, poor oral hygiene, periodontitis and enamel hypoplasia upper central incisor.


Illustrates the generalized bone loss and spacing.


Bilateral gynecomastia, patient 1.


Vascularized corneal opacity of left eye.


Graft showing brown lesion.


Total hand skin degloving injury. . Notes: (A) Before surgery.


Total hand skin degloving injury. (B) Superficial forearm vein with four branches.


Total hand skin degloving injury. (C) "Y shape" superficial forearm vein graft.


Appearance and function after surgery. . Notes: (A) Palm side at 2 years after surgery.


Appearance and function after surgery. (B) Dorsal side at 2 years after surgery.


Appearance and function after surgery. (C-F) Finger flexion function at 2 years after surgery.


The purulent oozing and stinky ulceration on the right side of labium minor measuring 5-8 cm.


The histopathological image stained with hematoxylin eosin X10 magnification.


The histopathological image stained with hematoxylin eosin X400 magnification.


The patients lesion healed with scar.


(a) Ultrasound showing a mixed echogenicity mass in the pelvis.


(b) Cut section of the retroperitoneal nodule showing gray-white whorled appearance.


MRI images showing endocranial mycotic abscess at day 41 and the result at the end of treatment with ISC, day 359.


TDM of ISC during one year of treatment showing an increment of plasma concentrations in correspondence with the improvement of intestinal GvHD (all blood samples were collected 12 hours after the last administration of the drug).


Thickening of facial skin with accentuated skin ruts and circumvolutions mainly in the frontal region, keloid scars, hypertrophic eyelid skin and lengthened eyelashes.


Hypertrophic eyelid skin and lengthened eyelashes.


A; Initial axial T2 of the dorsolumbar spine shows intramedullary expansion with T2 hyperintensity.


B; Significant regression in the follow-up image post-praziquantel treatment, with normalizing signal of the spinal cord.


Fluorescence in Situ Hybridization (FISH). A - FISH analysis showing EWSR1 gene rearrangement at the 22q12 locus. The 5' segment of the probe is orange and the 3' segment green.


Fluorescence in Situ Hybridization (FISH). In the inset, a magnified cell nucleus from the field shows a split probe on one chromosome (two distinctly separate orange and green dots) and an intact probe (arrow) on the other (fused orange and green signals); B - FISH analysis depicting NR4A3 (9q22.33-q31.1) gene rearrangement using another break-apart probe. The 5' segment of the probe is orange and the 3' segment is green. The arrows in the insets (1 & 2) point to the intact gene on one chromosome and a broken apart gene on the other chromosome.


Photographs of the right.


Left. Eyes.


Fundus autofluorescent (FAF) images of the right.


Left. Eyes are also shown. The FAF image shows a hypofluorescent lesion in the foveal and perifoveal areas consistent with bull's eye retinopathy. A prominent hypofluorescent lesion is visible in the left eye, indicating a marked atrophy of the retinal pigment epithelium layer. The bull's eye pattern of depigmentation is also evident on fundus photography and fundus autofluorescent images.


Adaptive optics scanning laser ophthalmoscope (AO-SLO) montage . The vertical SD-OCT images from both the right.


Adaptive optics scanning laser ophthalmoscope (AO-SLO) montage . Left. Eyes show loss of photoreceptor inner segment/outer segment junctions (moth eaten appearance).


Spectral-domain optical coherence tomography (SD-OCT). Images of both eyes.


Adaptive optics scanning laser ophthalmoscope (AO-SLO) montage from the left eye (A) matched with the corresponding red free image.


Magnified AO-SLO images (B,C) are also shown. (B) shows the area indicated by the white box on the montage. As shown in (B), disruptions in the cone mosaic, where cones were missing or lost, is apparent. These disruptions were not present in the normal subject. Additionally, in (B), cones appear to be asymmetrical in shape and size with variable brightness. Scale bar in.


Magnified AO-SLO images (B,C) are also shown. For comparison, (C) shows an age-matched normal retina in the same location. = 25 microm.


Correlation of structural and functional defects. Humphrey visual field (A) revealed a significant central defect.


Correlation of structural and functional defects. The adaptive optics scanning laser ophthalmoscope (AO-SLO) montage from the right eye (B) was matched with the infrared image.


Correlation of structural and functional defects. Images ,. The AO-SLO images ,. Cones in images ,. Scale bar in.


Correlation of structural and functional defects.


Correlation of structural and functional defects. Are magnified AO-SLO images of the locations of visual field defects (white boxes). , show the cone mosaic disruption and dark patchy lesions where cones are missing or lost. Were asymmetric in shape and size and exhibited variable brightness. = 25 microm. SR = superior retina; IR = inferior retina; NR = nasal retina.


Correlation of structural and functional defects. Images ,. Image D had the lowest cone density, and was lower than observed in a normal subject (F,G,H).


Correlation of structural and functional defects. Image D had the lowest cone density, and was lower than observed in a normal subject (F,G,H).


Correlation of structural and functional defects. Are images in the same location from an age-matched normal retina. The location and cone density for each figure is shown. Image D had the lowest cone density, and was lower than observed in a normal subject (F,G,H).


Clinical course and neutrophils count in relationship with drugs administration.


Corneal ulcer with infiltration and endothelial edema.


State of the cornea after deep anterior lamellar keratoplasty.


Magnetic resonance imaging showing a well-defined, enhancing lesion 9 mm in diameter at left posterior epidural space at D8 location displacing and compressing the cord.


Tumor cells showing positivity for vimentin, cytokeratins, fibrillary acidic protein and S-100 protein immunohistochemical stains.


Tumor cells show positivity for synaptophysin, negativity for epithelial membrane antigen and chromogranin immunohistochemical stains. MIB-1 labeling index approximately 7-10%.


(A) Gross thyroidectomy specimen including the tumor mass in the left lobe (prior to sectioning).


(B) Cut surface of the tumor with solid necrotic areas, cystic degeneration and extensive hemorrhage. The resected infrahyoid muscles are also present (yellow paint).


Microscopical aspect of thyroid angiosarcoma. Vasoformative area of the tumor with large uni- or multinucleated epithelioid tumor cells lining abnormal vascular channels containing papillary fronds.


Microscopical aspect of thyroid angiosarcoma. Or in solid sheets (HE, x200).


Immunohistochemical profile of thyroid angiosarcoma. Strong and diffuse membrane positivity for CD31 in solid areas (IHC, anti-CD31 antibody, x200).


Immunohistochemical profile of thyroid angiosarcoma. Total negativity for CD34 (IHC, anti-CD34 antibody, x200).


Immunohistochemical profile of thyroid angiosarcoma. Total negativity for D2-40 (IHC, anti-D2-40 antibody, x200).


Immunohistochemical profile of thyroid angiosarcoma. Positivity for CKAE1/AE3 in vascular channels (IHC, anti-CK AE1/AE3 antibody, x200).


Patient 2, axillary lymph node with AITL: (A) Partial nodal architecture effacement by a monomorphic lymphoid population and increased numbers of eosinophils, H&E, 100x.


(B) Neoplastic population of monomorphic follicular helper T cells with small to medium-sized nuclei and clear-to-pale cytoplasm in a polymorphous background of neutrophils and increased numbers of eosinophils with scattered CD30+ immunoblasts (circled, IHC not shown), H&E, 400x.


(C) Increased numbers of neoplastic helper T cells, CD4, 100x.


(D) Increased numbers of neoplastic follicular T helper cells (dim) with scattered neutrophils (strong), CD10, 100x.


(E) Increased numbers of neoplastic helper T cells, PD-1, 100x.


(F) Expanded dendritic meshwork, CD23, 100x.


Patient 3, cervical lymph node with T-ALL. (A) Relatively monomorphic infiltrate of neoplastic lymphocytes with irregularly shaped, hyperchromatic, small to medium-sized nuclei with inconspicuous nucleoli, 400x.


Patient 3, cervical lymph node with T-ALL. (B) CD34-negative, (CD34-positive blood vessels as internal control), 100x.


Patient 3, cervical lymph node with T-ALL. (C) CD10-negative, 100x.


Patient 3, cervical lymph node with T-ALL. (D) TdT-positive (nuclear), 100x.


Patient 3, cervical lymph node with T-ALL. (E) CD1a-positive, 100x.


Patient 3, cervical lymph node with T-ALL. (F) Ki-67 with 80% proliferation index, 100x.


Magnetic resonance imaging (MRI) of a lesion beside the left submandibular gland. The lesion demonstrates heterogeneous hyper-intensity on axial.


Magnetic resonance imaging (MRI) of a lesion beside the left submandibular gland. And coronary.


Magnetic resonance imaging (MRI) of a lesion beside the left submandibular gland. Fat-suppressed T2-weighted images, as well as on axial diffusion-weighted images.


Magnetic resonance imaging (MRI) of a lesion beside the left submandibular gland. Axial T1-weighted image (D) showing slight hypo-intensity.


Diagnosis of Legionella pneumophila infection using mNGS. (A) The majority of reads mapped to the L. Pneumophila genome with coverage of 28.12.


Diagnosis of Legionella pneumophila infection using mNGS. (B) The majority of reads mapped to the L. Pneumophila genome with coverage of 13.35.


(A,B) Gram stain of bronchoalveolar lavage fluid (BALF) with arrows indicating Legionella pneumophila, magnification x1,000.


(D) Gram stain of L. Pneumophila colonies (magnification x1,000).


Freckle-like brown macules and erythematous, indurated plaques with adherent scales.


Reticulate hyperpigmented and hypopigmented macules on neck and upper chest.


Reticulate hyperpigmented and hypopigmented macules on dorsa of hands.


Discoid-like patch over the scalp.


H&E stain, original magnification x400. . Note: Biopsy specimen taken from a hyperpigmented macule shows abundance of melanin pigment in keratinocytes and melanocytes and a few scattered melanophages. . Abbreviation: H&E, hematoxylin and eosin.


H&E stain, original magnification x200. . Note: Biopsy specimen taken from a hypopigmented macule shows reduced melanin and number of melanocytes. . Abbreviation: H&E, hematoxylin and eosin.


Clinical appearance of lesion.


Periapical radiograph of the right maxillary canine region.


Panoramic radiograph exhibiting radiolucent lesion.


Clinical appearance of lesion after 4 months of initial visit.


Hyperkeratotic and hyperplastic epithelium showing both endophytic and exophytic growth patterns. Rete ridges are narrow whereas superficial epithelial surface is broad. On the other side, normal gingival epithelium is appreciated.


Pseudorosette like cluster of cells around myxo-hyaline core (Pap, x400).


Hyaline globule with myxoid material surrounding cuboidal cells (Giemsa, x1000).


Clusters of cuboidal to columnar cells around central hyaline cores (Pap, x100).


Ovoid to spindle shaped cells with mild to moderate nuclear atypia and scanty cytoplasm, arranged discretely and also in some clusters in a myxoid background.


Mastectomy scar-left.


Tumour composed of spindled shaped cells with plump nuclei, arranged in intersecting fascicles and storiform pattern.


Mitotic figures and nuclear atypia-high power view.


Areas of myxoid change along with a capillary.


Tumour involving overlying skin.


Tumour cells stain positively for CD34.


Worm's-eye view.


Profile view.


CT and staining observations. . A. Axial CT after contrast administration in portal phase shows large mass that involved peritoneal surface without visceral organ affected.


CT and staining observations. B. Coronal reformatted CT after contrast administration in portal phase demonstrates inframesocolic and paracolic gutters mass with heterogeneous enhancement and few small foci inside (arrows).


CT and staining observations. C. Hematoxylin and eosin stain (20 x) shows glomus body consisting of uniform small, rounded cells with centrally placed round and pleomorphic nuclei. These cells are located around vessels.


CT and staining observations. D. Smooth muscle actin immunostain demonstrating strong cytoplasmic positivity.


Epithelial plaque and stromal abscessation in the right eye of a male red panda (case 1).


Cytology of affected corneal tissue in case 1, demonstrating filamentous fungal hyphae typical in appearance of Aspergillus spp.


Epithelial plaque and stromal abscessation in the left eye of a female red panda (case 2).


Histopathology of affected corneal tissue in case 2, demonstrating filamentous fungal hyphae, 100x magnification, oil immersion.


A: Photomicrograph shows an encapsulated parathyroid tumor (arrow) along with normal thyroid and parathyroid tissue (H&E 100X).


B: Photomicrograph shows fibrous bands dividing the neoplasm into lobules with cells arranged in sheets and trabeculae (H&E 100X).


Clinical and pathological differences between a benign parathyroid disease and a parathyroid carcinoma.


(A, B) The common axial position and oblique lateral position of the left breast with molybdenum targeting showing breast-occupying lesions. BI-RADS was used for classification into three categories.


(C) The surgical specimen was breast tissue with spinal skin and nipple (15 cm x 11 cm x 6.5 cm). The size of the spinal skin sample was 11 cm x 6.5 cm, and a nodule with a size of 8 cm x 6 cm x 5.5 cm was found under the nipple with multiple cuts. The nodules and surrounding mammary glands were clear. The nodules were gray and grayish yellow. Some areas showed cystic changes, some were solid and lobulated, and some were dark red, suggestive of necrosis.


Microphotographs showing the histopathological features of the tumor. (A) The tumor had loose and dense cell areas (H&E; 40x). The first arrow points to dense cell areas, and the second arrow points to loose cell areas.


Microphotographs showing the histopathological features of the tumor. (B) PT area (H&E; 40x).


Microphotographs showing the histopathological features of the tumor. (C) Fibroadenoma area around the malignant PT (H&E; 40x).


Microphotographs showing the histopathological features of the tumor. (D) Residual ductal epithelium (H&E; 200x).


Microphotographs showing the histopathological features of the tumor. (E) Mucinous background (H&E; 100x).


Microphotographs showing the histopathological features of the tumor. (F) Vascular rich area (H&E; 100x).


Microphotographs showing the histopathological features of the tumor. (G) Adipocytes (H&E; 400x).


Microphotographs showing the histopathological features of the tumor. (H) Odd megakaryocytes (H&E; 200x).


Microphotographs showing the histopathological features of the tumor. (I) Rosette-like cells (H&E; 100x).


(A) A few tumor cells were AE1/3 positive (original magnification: 200x).


(B) Tumor cells were diffusely positive for vimentin (original magnification: 200x).


Tumor cells were negative for E-cadherin.


S-100. (original magnification: 200x).


Tumor cells were diffusely, strongly positive for p16.


MDM2. (original magnification: 200x).


(H) Immunohistochemical staining for Ki-67 showed a labeling index of greater than 90% (original magnification: 100x).


(I) The FISH test results showed that MDM2 was not amplified.


Initial CT-scan with generalized brain edema.


Postoperative CT-scan demonstrating adequate bilateral decompression.


3D-reconstruction of postoperative CT-scan after bilateral decompression.


MRI after cranioplasty with bilateral titanium implants.


A follow-up PET-CT revealed the uptake of FDG only at the gallbladder (SUVmax 7.1).


Contrast abdominal CT showed an early enhanced mass in the gallbladder (arrow) and the enhancement persisted on the equilibrium phase, suggesting gallbladder cancer. (a) Early phase.


Contrast abdominal CT showed an early enhanced mass in the gallbladder (arrow) and the enhancement persisted on the equilibrium phase, suggesting gallbladder cancer. (b) Equilibrium phase.


MRI demonstrated that the gallbladder tumor (arrow) showed low signal intensity on T1-weighted images and slightly high on T2-weighted images. (a) T1-weighted images.


MRI demonstrated that the gallbladder tumor (arrow) showed low signal intensity on T1-weighted images and slightly high on T2-weighted images. (b) T2-weighted images.


The gallbladder tumor was macroscopically soft and whitish measured 7.5 cm x 5 cm in size.


Immunohistochemical examination revealed that the gallbladder tumor was not similar to the original nasal melanoma. HMB-45 staining was far less positive in the gallbladder tumor than in the nasal tumor. (a) Gallbladder tumor, HE staining.


Immunohistochemical examination revealed that the gallbladder tumor was not similar to the original nasal melanoma. HMB-45 staining was far less positive in the gallbladder tumor than in the nasal tumor. (b) Gallbladder tumor, HMB-45 staining.


Immunohistochemical examination revealed that the gallbladder tumor was not similar to the original nasal melanoma. HMB-45 staining was far less positive in the gallbladder tumor than in the nasal tumor. (c) Nasal tumor, HE staining.


Immunohistochemical examination revealed that the gallbladder tumor was not similar to the original nasal melanoma. HMB-45 staining was far less positive in the gallbladder tumor than in the nasal tumor. (d) Nasal tumor, HMB-45 staining (200x).


The clinical picture showing engorged swollen tongue.


The clinical photograph after the tongue swelling reduced.


Preoperative magnetic resonance imaging showing T1- weighted.


Fluid-attenuated inversion recovery. Images. The cyst content demonstrates slightly higher intensity than regular cerebrospinal fluid on fluid-attenuated inversion recovery images, containing a mucous-like nodule with isointensity on T1WI and mild high intensity on T2WI.


Uncal herniation is observed at the level of the mesencephalon (d).


Intraoperative presentation of the cyst. The cyst is covered by a thin whitish membrane and contains white turbid fluid.


Postoperative course is organized as a timeline. The patient experienced a tonic-clonic seizure 9 h after surgery. Lumbar puncture and electroencephalogram were performed on postoperative day 1. The seizure was finally controlled with levetiracetam 4000 mg, Fosphenytoin 7.5 mg/kg, Lacosamide 200 mg, and propofol 0.3 mg/kg/h.


(a and b) Postoperative magnetic resonance imaging shows cyst shrinkage and disappearance of the mass effect.


Photomicrograph of immunohistochemistry showing positive for AFP (x400).


(A) Haematoxylin and eosin (H&E) stain of the neck nodal mass showing nonkeratinized squamous cell carcinoma (x200) in 2000.


(B) Strong and diffuse p16 IHC stain of the tumour present in cervical lymph node from 2000.


FDG-PET from 2011 showing multiple FDG avid osseous metastatic lesions.


Intraosseous metastasis of nonkeratinizing squamous cell carcinoma in 2011 (H&E x100).


P16 IHC stain of the bone metastasis from 2011.


High-risk HPV ISH of the bone metastasis from 2011 showing positive intranuclear blue stain.


MRI abdomen pelvis, T2 axial image, important pelvic cavity invasion with vesicular like lesions, englobing the uterus, the left iliac region with involvement of the posterior fascia of the left rectus abdominis muscle. .


Pathologic findings of growing teratoma (right bottom) in contact with the striated muscle of the rectus abdominis muscle (HES x50).


Hemispherical nodule in the subungual region of the right great toe.


Fine needle aspiration smear showing benign plump oval to spindle-shaped cells in groups, sheets and cohesive clusters in a background of myxoid material and insert showing the histopathological section, confirming the cytological findings (Pap, x400).


Slit lamp photograph of the right.


Left. Eyes. When a slit beam of light was shown to the limbus from one side, the shape of light reflex seen in the opposite side indicated steepening of the inferior cornea (white arrows in each figure) just above the thinning.


Perioperative findings. At referral, a corneal perforation plugged by iris superotemporally. Arrows) and a flat anterior chamber in the left eye are seen on slit-lamp examination.


Perioperative findings. At referral, a corneal perforation plugged by iris superotemporally. And ultrasound biomicroscopy.


Perioperative findings. Slit-lamp examination . *) completely covers the corneal defect.


Perioperative findings. AS-OCT. Show that 5 days after corneal grafting,. The donor corneal tissue. *) completely covers the corneal defect. Arrows) on the host cornea.


Perioperative findings. Seven weeks postoperatively, slit-lamp examination (e) shows peripheral movement of the anterior edge of the patch graft and exposure of the previously perforated area (arrow).


Perioperative findings. Arrows) on the host cornea. #). An AS-OCT (f) image shows that stromal scar tissue (arrow) covered by corneal epithelium has closed the corneal defect.


A; Monochromatic green fundus photograph of the right eye showing optic disk pallor, peripapillary atrophy, arteriolar narrowing, and typical bone spicules involving the mid-peripheral retina.


B; Fundus autofluorescence photograph of the right eye. Note the extent of the atrophy in the mid-periphery.


C; Spectral domain optical coherence tomography revealing a cystic macular edema associated with a disruption of the Bruch membrane/retinal pigment epithelium complex overlying a pigmentary epithelium detachment, with a vascular structure emanating from the deep capillary plexus, apparently connected with the subretinal pigment epithelium space. Also note the presence of an epiretinal membrane.


D-f Optical coherence tomography angiography images (3 x 3 mm) and corresponding B-scans. D; Optical coherence tomography angiography deep capillary plexus segmentation showing a high-flow vessel infiltrating the outer retinal layers (arrow).


E; Outer retinal layer segmentation showing a tuft-shaped high-flow lesion, characterized by a retinal-retinal anastomosis.


F; Choriocapillaris segmentation revealing the tuft-shaped lesion.


Postoperative (day 2) photograph showing inversion of Bell's phenomenon in both eyes following right frontalis sling-suspension surgery.


Change in tumor pattern. MRI before chemotherapy.


Change in tumor pattern. , MRI after chemotherapy.


Change in tumor pattern. , CT scan before chemotherapy.


Change in tumor pattern. And CT scan after chemotherapy.


Calcified primary tumor (standard X-ray).


The magnetic resonance imaging of the brain revealing a large mass in the posterior horn of the right lateral ventricle attached by a pedicle.


Magnetic resonance imaging on admission. The two mass lesions in the left body of the lateral ventricle and the right cerebellum hemisphere were isointense on T1-weighted images.


Magnetic resonance imaging on admission.hypointense on T2-weighted images.


Magnetic resonance imaging on admission. The lesions were enhanced after gadolinium-diethylenetriamine pentaacetate injection (c and f).


Two weeks following hospital admission, the right cerebellum lesion almost disappeared (a).


Two weeks later, new enhanced lesions emerged in the choroid plexus of the fourth ventricle (b).


Enlarged image of the left body of the lateral ventricle lesion (c).


Intraoperative neuroendoscopic image showed irregular surface in the lateral ventricle. Many small particles were floating in the cerebrospinal fluid, which was suggestive of leptomeningeal dissemination.


(a-c) After steroid pulse treatment, the lesions almost disappeared.


(a-c) After 12 months, all the lesions had resolved and had not recurred.


Magnetic resonance imaging.


Skin incision.


The nodule.


After excision.


Post-operative healed wound.


Histopathology slide:Photomicrograph of HPE slide showing HNE stained at x40 showing ill-circumscribedlesion composed of spindle-shaped cells in a fibrous stroma background.


Histopathology slide:Photomicrograph of HPE slide showing HNE stained at x100 showing bland spindle-shaped cells with abundant collagen in a fibrous stroma background.


Haematoxylin, and . Eosin staining of a tumour section. X10 magnification.


Haematoxylin, and . . X40 magnification). The pathological diagnosis was adrenal angiogenic malignant tumour with massive necrosis.


Immunohistochemistry showed positive reactivity for CD31.


CD34. In the tumour cells, indicating that they were of endothelial origin (x40 magnification).


Seven months after operation, positron emission tomography/computed tomography was performed. (A, B) The soft tissue mass in the operative area was accompanied by an abnormal increase of fluorodeoxyglucose metabolism (maximum standardized uptake value, 21.0).


Seven months after operation, positron emission tomography/computed tomography was performed. (C, G) after two cycles of chemotherapy.


Seven months after operation, positron emission tomography/computed tomography was performed. (D, H) after six cycles of chemotherapy.


Seven months after operation, positron emission tomography/computed tomography was performed. (E, F) The larger lymph nodes were located in the 8R area (4.2x 1.9 cm, maximum standardized uptake value, 7.9). Computed tomography during treatment showed that the tumour lesions disappeared in the arterial phase and the mediastinal and mediastinal-hilar lymph nodes were significantly reduced or had disappeared.


Annular plaque with erythematous to violaceous, raised, indurated periphery and centrally atrophied plaque over the left lower limb involving the entire knee joint and posterior aspect of the upper leg.


Post-treatment photograph showing flattening of plaque and reduction of erythema with center of the plaque showing three hypertrophic scar marks.


Colonoscopy. . Notes:. Hepatic flexure of colon malignant lesions.


Colonoscopy. Rectal malignant lesions.


Colonoscopy. A large number of colorectal polyps.


Surgical specimens. . Notes:. Malignant tumor found in the hepatic flexure of the colon.


Surgical specimens. A large number of polyps were found in the rest of the colon, and ,rectum.


Surgical specimens. Malignant tumor found in the rectum.


Histopathology. . Notes:. Carcinoma infiltrating the serosa, with ulcerative moderately differentiated adenocarcinoma.


Histopathology. A tubular-villous adenoma colonic polyp.


Histopathology. Lymph node metastasis. Scale bar 100 mum; magnification x400.


Chest x-ray:. Posteroanterior.


Lateral. Small calcified nodes were present in the inferior right hilum and subcarinal chain (indicated by the arrow). No suspicious pulmonary nodules nor infiltrates were identified.


The 2.0x1.0 cm mildly erythematous thin plaque with a yellow crust.


Preoperative intraoral photograph showing swelling over the left molar region.


Preoperative radiograph (orthopantomogram) showing the multilocular lesion extending till the lower border and the subsigmoid region, with multiple impacted teeth.


Exposure of the tumor from the buccal side.


Complete enucleation of the tumor.


Primary closure with 3-0 vicryl sutures.


Postoperative radiograph at 1-year follow-up showing good bone growth without recurrence.


Prosthesis given to the patient posthealing as a space maintainer.


Strands and cords of epithelium in ectomesenchymal tissue stroma.


A) When the forceps picked up the epiretinal tissue, the inferior temporal retinal artery was accidentally involved (yellow arrow) and broken off.


B) The broken artery (white arrows) was floating and pulsating in the vitreous space. Microscopic bleeding at the broken end and oozing from the retinal cleft that had once enfolded the artery were observed.


A) Preoperative: Path of the inferior temporal artery is indicated by white arrows.


B) Postoperative: The artery (white arrows) is broken and curled up above the optic disc.


C) Pattern deviation of visual field perimeter (Humphrey Field Analyzer, 30-2 program; Carl Zeiss Meditec, Inc. , Dublin, CA, USA) obtained postoperatively from the patient's left eye. The superior nasal field was lost.


Preoperative and postoperative imaging features of the patient. T2-weighted MRI axial.


Preoperative and postoperative imaging features of the patient. Coronal.


Preoperative and postoperative imaging features of the patient. Images show a solid tumor arising from the trigeminal nerve (red arrow) with heterogeneous contrast-enhanced.


Preoperative and postoperative imaging features of the patient. Axial MRI images reveal gradual enlargement of the tumor.


Preoperative and postoperative imaging features of the patient. Destruction of the adjacent skull base bone is shown in the oval.


Preoperative and postoperative imaging features of the patient. Postoperative CT shows subtotal removal of the tumor.


Preoperative and postoperative imaging features of the patient. Follow-up CT scanned 3 weeks after surgery shows tumor recurrence.


Hematoxylin-eosin and immunohistochemical staining of the tumor tissue. Histological staining shows a high cell density with eosinophilic cytoplasm, and ,prominent nucleolus.


Hematoxylin-eosin and immunohistochemical staining of the tumor tissue. Positive expression of EMA, VIM, CD99, synaptophysin, and ,BRG1, respectively.


Hematoxylin-eosin and immunohistochemical staining of the tumor tissue. Negative expression of desmin and INI-1 (at 200 magnification).


Chest x-ray showing homogeneous hypotransparency in the medial third of the left lung field.


Frosted branch angiitis in a case of sympathetic ophthalmia.


This figure shows a T2W MRI midsagittal section of the c-spine in which a posteriorly originating hypointense epidural collection is seen suggesting a hyperacute epidural hematoma.


This figure shows a T2W MRI sagittal section of the c-spine in which an epidural collection mainly located at the level of C5-C6 is present with signs of incipient cord compression.


Exploration of the perianal pilonidal sinus in the prone position at 7 o'clock.


Removal of the hair and curation of the cavity.


Optical coherence tomography (OCT) scan showing retinal pigment epithelium (RPE) repair process in the right eye during anti-VEGF treatment. 1 month after treatment: a subfoveal OCT scan shows splitting of the RPE (yellow arrow) with persistent well defined subretinal hyperreflective material (yellow star) and subretinal fluid; b OCT scan superior to the fovea shows continuity of the hyperreflective line attributable to the RPE (white arrow) and envelopment of the hyperreflective material (white star). 9 months after initiation of treatment; c Subfoveal OCT scan shows further consolidation of the hyperreflective material with persistent splitting of the RPE (yellow arrow); d OCT scan superior to the fovea shows reducing subretinal fluid and integrity of the RPE (white arrow).


Fundus photograph showing a whitish, nodular, endophytic mass lesion with multiple vitreous seedings.


B-scan ultrasonography showing mass lesion with moderate-to-high internal reflectivity with calcification.


Orbital computed tomography. . Notes: (A) Coronal section.


Orbital computed tomography. (B) Transverse section. Showing a mass lesion in the right lacrimal gland, with regular borders and internal heterogeneity, determining an inferior and medial deviation of orbital structures.


Pleomorphic adenoma with a malignant component of myoepithelial carcinoma (right inset). . Notes: Peripherally, there is infiltration by chronic lymphocytic leukemia/small lymphocytic lymphoma (left inset).


Computed tomography imaging showing hyperdense hemorrhagic lesion in the left temporal horn of lateral ventricle with hemorrhage in left lateral ventricle.


Contrast-enhanced angiography showing evidence of well-defined, strongly homogenous, enhancing polypoidal intraventricular mass lesion both in arterial phase and venous phase with hemorrhage in left lateral ventricle.


A cauliflower-like vascular mass in the temporal horn of lateral ventricle with an attachment to the choroid plexus.


Noncontrast computed tomography showing dilated both lateral, third and fourth ventricles suggestive of moderate communicating hydrocephalus.


(a) Papillary fronds lined by single layer of cells (H and E, x40). (b) Delicate fibrovascular connective tissue fronds covered by single layer of uniform cuboidal to columnar epithelial cells with round to oval, basally situated nuclei (H and E, x200).


Fungal blood culture demonstrating white to tan-colored, velvety and flat colonies with red soluble pigment.


Lactophenol cotton blue staining from fungal blood culture demonstrating septate hyphae and smooth conidia aloft phialides which are borne to metulae.


Contrast-enhanced computed tomography of the whole abdomen demonstrating small hypodense lesion with a thin enhancing rim at the spleen and extensive intra-abdominal lymphadenopathy.


Aspirate smear showing mature adipocytes admixed with benign appearing spindle cells and few traversing capillaries (Pap, x100).


26-year-old female with chest pain and breathlessness diagnosed with pleural and peritoneal mesothelioma. Chest X-ray poster anterior view reveals opaque right hemithorax with areas of radiolucencies in the right mid zone (horizontal arrow). Right costophrenic angle and right hemi diaphragm silhouette is obliterated by soft-tissue opacity. There is no evidence of any calcification or bone destruction. Right side shows mild tracheal and mediastinal shift. Left lung field and costophrenic angle on the left side are clear.


26-year-old female with chest pain and breathlessness diagnosed with pleural and peritoneal mesothelioma. Post-contrast CT coronal view shows contiguous rind-like soft-tissue attenuation pleural mass lesion encasing right hemithorax and scalloping of liver (horizontal arrow). Right side shows axillary lymphadenopathy (vertical arrow).


26 year-old female with chest pain and breathlessness diagnosed with pleural and peritoneal mesothelioma. Post-contrast CT scan axial view shows pleural-based mass lesion (horizontal arrow) causing scalloping of the liver parenchyma with loss of extra pleural fat planes along the right lateral chest wall. No rib destruction is noted.


26-year-old female with chest pain and breathlessness diagnosed with pleural and peritoneal mesothelioma. Post-contrast CT scan axial view shows soft-tissue attenuation pleural mass lesion completely encasing the right lung which shows consolidation collapse. Right lateral chest wall shows loss of extra pleural fat plane (horizontal arrow). However, no definite invasion of intercostal muscles is seen.


26-year-old female with chest pain and breathlessness diagnosed with pleural and peritoneal mesothelioma. Post-contrast axial CT sections from the pelvis show homogenously enhancing soft-tissue attenuation mass lesion (horizontal and vertical arrows) in the pelvis, abutting the small and large bowel loops without causing any obstruction. Ascites is also noted.


26-year-old female with chest pain and breathlessness diagnosed with pleural and peritoneal mesothelioma. (a) Histopathology slide of the biopsy specimen. Hematoxylin and eosin staining of the lung biopsy sample (x400) shows malignant epithelial cells in acinar pattern (vertical arrow) infiltrating the lung tissue in a linear fashion (horizontal arrow).


26-year-old female with chest pain and breathlessness diagnosed with pleural and peritoneal mesothelioma. (b) Biopsy sample stained for Wilms' tumor protein shows nuclei positive (horizontal arrows) for the protein (x1000), which confirms the diagnosis of mesothelioma.


Photographic progression demonstrating the dramatic weight gain of our patient over 18 months. 18 months prior to presentation.


Photographic progression demonstrating the dramatic weight gain of our patient over 18 months. Just prior to presentation with her mother.


Photographic progression demonstrating the dramatic weight gain of our patient over 18 months. At admission while in the ICU.


A; Adrenal gland on cross sectioning shows several discrete, deeply red-brown nodules, consistent with PPNAD.


B; Golden brown pigment with the cytoplasm of cortical cells from the adrenal gland are seen on microscopic examination, imparting the typical pigment seen on gross examination with PPNAD (H&E stain, original magnification 400x).


Initial slit-lamp examination of the left eye. Diffuse disseminated pigmentation in the conjunctiva can be seen around the cornea.


Rapid histopathological analysis of initial biopsy. There was no excess melanocyte proliferation in the epithelial layers. Thus, it was diagnosed as primary acquired melanosis (PAM) with reactive lymphoid hyperplasia and without atypia. Low magnification.


Rapid histopathological analysis of initial biopsy. There was no excess melanocyte proliferation in the epithelial layers. . High magnification).


Seven years after excisional biopsy. No change was observed on the pigmentation in the conjunctiva.


Fourteen years after the excisional biopsy. Black tumor expanding from the fornix and disseminated pigments at bulbar and palpebral conjunctiva can be seen.


Histopathological specimen of the conjunctival tumor (H&E staining). Tumor was a conjunctival malignant melanoma.


Linearly arranged nevus comedonicus extending from left anterior chest to left upper back.


Nevus comedonicus involving left axilla complicated with multiple abscesses.


Nevus comedonicus extending over the back.


Local flap planned for transposition.


Wide local excision of the left axilla skin and subcutaneous fat, down to the level of deep fascia.


Axillary defect was resurfaced with a transposition flap from left parascapular region and the donor site skin grafted.


Anterior chest wall region was closed primarily and healed well.


Posterior chest wall region covered with a split-thickness skin graft, which has healed well.


Axillary flap settled well on long-term follow-up.


A; Color fundus photograph 5 weeks after initial presentation demonstrating inferotemporal pigmented chorioretinal scar (white arrow) with improvement of subretinal fluid revealing active retinochoroiditis adjacent to the old toxoplasmosis scar. B; OCT demonstrating active TRC lesion with overlying vitreous cell. C; Macular scan demonstrating resolution of subfoveal fluid. +Image copyright holder is the Department of Ophthalmology and Visual Sciences at the University of Michigan W. K. Kellogg Eye Center. OCT, optical coherence tomography.


Patient's photograph shows left lower limb polymelia.


Radiograph of the patient reveals a normal left hip joint with an accessory left underdeveloped limb. The accessory limb has rudimentary femur falsely attaching to normal acetabulam of left hip joint. Both rudimentary femur and the tibia form a false knee joint. Distal most end of the accessory limb shows a single false digit with a single curved false metatarsal.


Computerized tomography axial section.


The coronal reformatted image. Showing the obstructed left kidney (white arrows) distended with air and fluid. The parenchyma is markedly thinned out.


Periocular petechiae and diffuse subconjunctival hemorrhage found in both the eyes.


Right eye.


Left eye of a patient who underwent photorefractive keratectomy and presented with bilateral paralimbal infiltrates outside the optical zone at day one post-operatively.


Complete resolution of infiltrates in the. Right eye.


Left eye of a patient who developed bilateral paralimbal infiltrates after photorefractive keratectomy.


Tc-99m MIBI SPECT-CT study of the chest. Cross sectional images revealed a Tc-99m MIBI avid soft tissue lesion in the anterior mediastinum adjacent to ascending aorta (image (a) red arrow), which was confirmed to be a parathyroid adenoma on histopathological examination.


Tc-99m MIBI SPECT-CT study of the chest. Multiple well-defined marginated expansile lytic lesions (Brown tumors) are noted in the ribs (images (b-d); arrows).


Tumor extending from distal aspect of 37 to uvula.


Photomicrograph showing epithelial component around the glandular spaces and arranged in solid nests surrounded by spindle component. (H&E stain, x400).


Immunohistochemistry revealed strong nuclear positivity of tumor cells for Transducer-like enhancer of split 1 (TLE1) in both spindle and epithelial component (IHC stain, x100).


Immunohistochemistry revealing Epithelial Membrane Antigen (EMA) positivity in the cuboidal epitheloid cells and negativity in the spindle cells (IHC stain, x100).


Immunohistochemistry revealing vimentin positivity in the spindle cells and negativity in the cuboidal cells (IHC stain, x400).


Immunohistochemistry revealing Bcl-2 positivity in the spindle cells (IHC stain, x100).


External photograph showing bilateral eyelid swelling and severe conjunctival injection.


External photograph showing macroscopically visible giant papillae in the right lower tarsal conjunctiva.


Basal abdominal CT scan revealing an oval-shaped, homogenous mass, with muscular density, developed in the subcutaneous tissues of the anterior abdominal wall. The mass is well-circumscribed with regular limits clearly differentiated from the surrounding structures.


Post-contrast abdominal CT scan (later phase) revealing a mild and homogeneous enhancement with a few scattered liquid chambers.


Operative view showing a well-defined subcutaneous mass easily enucleated through a selective incision in the right iliac fossa.


Macroscopic view of the resected specimen showing an encapsulated solid tumor with heterogeneous appearance and the presence of hemorrhagic as well as mucoid changes.


Microscopic findings: Tumor proliferation composed of spindle-shaped cells with a benign appearance. No evidence of mitosis or cytonuclear atypia (hematoxylin-eosin staining, original magnification x10).


Immunohistochemical study showing strong and diffuse staining for the S-100 protein. (immunohistochemistry, original magnification x40).


Astrocytic cells dispersed in fibroblastic stroma (Hematoxylin & Eosin stain A:X40 B:X100).


GFAP (+) Astrocytic cells, 100X.


CD34 (+) fibroblastic cells, 100X.


S-100 (+)Astrocytic cells (+), 100X.


Gross findings of the pericardial sac.


Epicardium. During surgery. Multiple white nodules (arrows in A) and plaques (arrowheads in B) were identified on the surface of the thickened pericardial sac and the epicardium, respectively.


Ultrasonographic evaluation of disseminated neoplastic lesions on the pleura. Small nodules of 1-1.3 mm size on the surface of the pleura, exhibiting homogeneous low echogenicity inside with a hypoechoic structure, were identified, which had not been noted at the early stage of the disease, via detailed ultrasonographic examination using a high frequency linear probe (A, arrows).


Ultrasonographic evaluation of disseminated neoplastic lesions on the pleura. The adjacent normal pleurae were delineated as finely hyperechoic linear structures (B, arrowheads).


Ultrasonographic evaluation of disseminated neoplastic lesions on the pleura. The nodules gradually increased in size and number on the parietal pleura. Arrows) as well as the visceral pleura.


Ultrasonographic evaluation of disseminated neoplastic lesions on the pleura. Diaphragm. Arrows). Arrowheads, parietal pleura; TW, thoracic wall; PE, pleural effusion; DP, diaphragm; PL, lung; LIV, liver. Scale bar = 0.5 cm.


Pre-operative radiologic finding-Anteroposterior.


Oblique. View X-rays of the right foot: Multiple loose bodies around the right first metatarsophalangeal joint at the first web space with preservation of the articular surfaces.


Computed tomography (CT) scan pre-operative study-CTsagittal.


Axial. Section at the first metatarsal space shows a well-defined soft tissue mass with multiple curvilinear and punctate calcified densities inside located at the first metatarsal head.


Intraoperative image-Intraoperative aspect of the first web space before.


After. Resection of the tumor.


Intraoperative macroscopic aspect-The joint contains multiple osteochondral bodies and hypertrophied synovial tissue nodules.


MRI of the right shoulder showing the tumor extent at the time of initial diagnosis in proton density (PD)-weighted.


T1-weighted contrast-enhanced. Sequences. An inhomogeneously enhancing, partially fibrotic tumor (white arrows) originating from the right thoracic wall is clearly visible consistent with a desmoid-type fibromatosis.


T2-weighted MRI of the right shoulder after second tumor resection and prior to bevacizumab treatment.


As well as 6 weeks.


4 months.


14 months. After treatment initiation, showing a continuous tumor shrinkage over time (white arrows).


Typical facial appearance of twelve-year-old female patient with osteogenesis imperfecta.


Slit-lamp examination; nasal limbal gelatinous mass with inferior corneal infiltration.


A) One month later, the lesion had decreased.


B) At the third month, CIN had disappeared.


A) Slit-lamp examination: a gelatinous temporal conjunctival mass (7x10 mm) with dilated superficial vessels without corneal involvement is exposed.


B) Abduction limitation in right eye.


Anatomopathological study: moderately differentiated squamous cell carcinoma was confirmed; tumour cells present in corion.


Tumor progression involving ocular globe tissues and soft periorbital structures.


Imaging of ultrasound and MRI examination of breast. . Notes: (A) A low echo mass of 8x5 cm2 can be seen in the left axilla by ultrasound examination. The boundary was unclear, and the shape was irregular.


Imaging of 18 F-fluorodeoxyglucose (FDG) PET/CT. . Notes: The saccharometabolism of the left axillary mass was in homogeneously increased and the lesions were not clear with the boundary of musculi teres minor, subscapalaris and the scapula on the left. Left side vertical images (transverse section of the chest): The images from top to bottom are CT plain scan, PET plain scan, and the synthesis of PET and CT images. Upper right images (front projection of human body): The images from the second to the fourth are CT plain scan, PET plain scan, and the synthesis of PET and CT images. Lower right images (side projection of human body): The images from the second to the fourth are CT plain scan, PET plain scan, and the synthesis of PET and CT images.


Image of core needle biopsy histologic diagnosis using hematoxylin and eosin staining (scale bar =2,000 mum; original x100). . Note: Glassy degeneration and focal vascular hyperplasia can be seen in the fibrous tissue.


Intraoperative photo of the mass. . Notes: (A) The complete appearance of the tumor.


Intraoperative photo of the mass. (B) Tumor section image. The mass was large (12x11 cm2), and the section of the mass was presented as fibrous tissue interleaving.


Image of paraffin pathology histologic diagnosis using hematoxylin and eosin staining (scale bar =2,000 mum; original x100). . Notes: (A) An abundance of spindle cells is evident.


Image of paraffin pathology histologic diagnosis using hematoxylin and eosin staining (scale bar =2,000 mum; original x100). (B) Tumor involvement in striated muscle is seen.


Chronic osteomyelitis. A 48-year-old man with painless swelling of left foot. Frontal radiograph shows moth eaten area of destruction involving tarsal (white arrow) and metatarsals (black arrow) with patchy erosions and mild soft tissue swelling (yellow arrow).


T2 weighted fat suppressed sagittal.


T1W fat suppressed postcontrast sagittal images show extension of inflammation proximally up to distal leg with ankle joint effusion (yellow arrow) and synovial thickening. "Dot in circle" sign (white arrow) is seen in postcontrast image.


Histopathology slide prepared using lactophenol cotton blue shows septated conidia which is curved at 3rd cell from the base (yellow arrow) confirming the diagnosis of curvularia lunata (eumycetoma sp. ) x1500.


(a) Photograph showing large solid non-mobile epibulbar mass with injected surrounding conjunctiva.


(b) Photograph showing scarring and pannus at the site of excision four months postoperatively.


(a): Photomicrograph of limbal mass at low magnification. Note the extensive inflammatory infiltrate in the substantia propria and large histiocytic with pale staining cytoplasm (hematoxylin and eosin; original magnification x4).


(b): an aggregate of histiocytes at high magnification. Note the emperipolesis of eosinophils (arrow), lymphocytes and polymorphonuclear leukocytes within the histiocytes (hematoxylin and eosin; original magnification x20).


(c): note positive S-100 staining of the histiocytes (DAB chromogen; original magnification x40).


First preoperative MRI shows a cystic plexus papilloma in the left parietal region. An enhanced ring can be visualized after gadolinium. Histological diagnosis revealed a CPP grade I WHO.


Post operative CT scan showed the partial removal of the extra ventricular cystic lesion.


A new MRI, after 7 months, showed a new cystic lesion in the cerebellar vermis with an eccentric nodule, in the temporal lobe and a recurrent multiloculated lesion in the left parietal region. Histological diagnosis was CPC grade III WHO.


A. The lesion in the sigmoid colon.


B. An image with a fishbone penetrates to the peritoneal cavity.


A,b. Dystrophic nail changes; pitting, onychorrhxis and leukonychia.


Picture taken during flare of erythema multiform: showing maculopapular, blanchable, dusky and red in color that was non-itchy rash and involved both hands, forearms, body and lower limbs.


Picture of left forearm two months after discharge: resolution of erythema multiform rash with residual skin hyperpigmentation.


Picture of right forearm two months after discharge from the hospital: again, resolution of erythema multiform rash can be appreciated.


Inflammation of the anterior dorsal tongue is evident.


Low magnification of the lip specimen reveals intact normal arranged epithelium lining. Directly below are dilated blood vessels in the superficial lamina propria surrounded by sparse lymphocytic infiltrate scattered throughout the area. In the deep reticular zone there are numerous large and small nests of non-caseating granulomatous infiltrate with scattered inflammatory cells.


Medium magnification of the lip specimen reveals large and small clustered nest of non-caseating granulomatous infiltrate consisting of epithelioid histiocytes with no giant cells. Scattered chronic inflammation and dilated blood vessels at the superficial lamina propria and surrounding granulomatous nest.


Low magnification of anterior tongue specimen reveals numerous and extensive lymphocytic inflammatory response surrounding blood vessels and interlaced between are nests of non-caseating granulomatous infiltration within the reticular zone.


(a, c) Sagittal and coronal T1-weighted MRI brain scan showing a sphenoidal sinus tumor.


(b, d) Sagittal and coronal contrast-enhanced T1-weighted MRI showing a sphenoidal sinus tumor with heterogeneous contrast enhancement.


(a, b) Sagittal and coronal contrast-enhanced T1-weighted postoperative MRI showing complete resection of the tumor.


(A) Periapical radiography of 21 region does not show any significant bone lesion.


(B) Maxillary occlusal radiography does not reveal any significant bone changes.


(C) Orthopantomography does not show any bone lesion either.


On close inspection of the back, multiple closely grouped papular lesions can be appreciated over the back, giving a rough, cobblestone-like texture to the skin.


Special staining with colloidal iron showed amorphous blue-colored mucin deposition throughout the dermis, which in correlation with the histopathological picture is diagnostic for scleromyxoedema. Counter stain used: Hematoxylin (Colloidal iron stain: x10).


Specimen after opening the cyst.


Squamous metaplasia in the cyst.


Langerhan's giant cells seen in tuberculosis lymphadenitis.


Fundus pictures of the right.


Left eye. Showing bilateral optic disc edema suggestive of papilledema.


Preoperative brain computed tomography scan (a) of the patient revealed a large heterogeneous mass at right parieto-occipital region with some vasogenic edema that produces a 0.5 cm midline shift of the brain.


On the brain magnetic resonance imaging, we found a solid-cystic hyper-intense mass on T2 sequences.


That it is brightly enhanced after injection of gadolinium.


Follow-up brain magnetic resonance imaging with gadolinium revealed no remnant of tumor (d).


CE- MRI brain of Case 2. T1W image axial view.


CE- MRI brain of Case 2. T1W contrast image axial.


CE- MRI brain of Case 2. Sagittal.


CE- MRI brain of Case 2. Coronal. Views.


CE MRI brain of Case 3. T1W image axial view.


CE MRI brain of Case 3. T1W contrast image axial.


CE MRI brain of Case 3. Sagittal.


CE MRI brain of Case 3. Coronal. Views.


CE-MRI brain of Case 4. T1W image axial view.


CE-MRI brain of Case 4. T1W contrast image axial.


CE-MRI brain of Case 4. Sagittal.


CE-MRI brain of Case 4. Coronal. Views.


Intraoperative image of case 4. Green-colored pus of mucormycosis.


Subungual hyperkeratosis (arrow) , onycholysis, onychomadesis, and a transparent serous exudate emanating from the toenail bed (circle).


Subungual hyperkeratosis (arrow).


Fingernail changes included dark pigmentations, Beau's lines, and subungual hyperkeratosis (A).


The patient's hands became desquamated and red (B).


Dysesthesia symptoms remained, and all the toenails exhibited defects (arrow). Or deformities (circle). At a 24-month follow-up.


Or deformities (circle). At a 24-month follow-up.


Fingernail changes improved at a 24-month follow-up (A).


The patient's hands returned to normal (B).


Transabdomen grayscale US image shows a heterogeneous lesion demonstrating solid and cystic portions arising from the left ovary.


Transvaginal US image shows a complex mass arising from the left ovary with solid.


Cystic components. Color Doppler examination of the same mass demonstrates high vascularity in the solid portion of the left ovarian mass.


Axial CT images with IV contrast demonstrate the presence of a heterogeneous mass in the left adnexa (arrows) with macro calcifications and enhancement of the solid components.


Hematoxylin and eosin-stained tissue (400x) displaying branching papillae with atypical cytologic features including nuclear groves, clearing, overlapping, and enlargement, consistent with papillary thyroid carcinoma arising in a struma ovarii.


Multiple crusted plaques over trunk.


Multiple crusted plaques over buttocks.


Keratoderma blenorrhagicum - soles.


Circinate balanitis.


Histopathology showing marked hyperkeratosis, focal parakeratosis, with spongiosis, irregular rete ridges and few micro abscesses in the epidermis and predominantly lymphocytic infiltrate in perivascular area in the dermis.


Finger nails showing coarse pitting, yellowish discoloration, transverse ridges and subungual hyperkeratosis.


X-ray hip joint (AP view) showing reduced joint space.


A; Patient prior to orbital decompression.


B; Following decompression surgery, botulinum toxin injection, and eyelid repair for treatment of upper eyelid retraction.


A; Coronal T1-weighted contrast-enhanced MR scan of orbits demonstrates irregular enlargement of the right lacrimal gland.


B; Normal extraconal muscles.


A; Coronal computed tomography of the brain without contrast enhancement at presentation demonstrates normal extraconal muscles.


B; Coronal computed tomography of the brain without contrast enhancement 2 years after presentation demonstrates irregular enlargement of the left inferior rectus.


Upper abdomen with diffuse dilatation of the esophagus, stomach and small bowel.


Lower abdomen with diffuse dilatation of the large bowel.


Intraoral view showing buccal cortical expansion with ulceration on the palatal aspect.


Orthopantomogram showing ill-defined radiolucent lesion i. r. t. right maxillary posterior region with displaced roots of the regional teeth.


PAS-positive cytoplasmic granules. Inset: diastase sensitive granules (x400).


Mild cytokeratin (CK-8) immunoreactivity in the tumor islands (x400).


Moderate cytokeratin (CK-19) immunoreactivity (x400).


Vimentin immunoreactivity localized to the fibrous connective tissue stroma (x400).


Lesion (white solid arrows) of the right petrous apex as viewed on different axial MRI sequences. (A) T2-weighted imaging shows a slightly heterogeneous lesion with parts isointense to white or gray matter. The dotted arrow shows the internal carotid artery.


Lesion (white solid arrows) of the right petrous apex as viewed on different axial MRI sequences. (B) The lesion is slightly hypointense to brain on T1 VIBE image and demonstrates slight peripheral enhancement following injection of gadolinium contrast.


Lesion (white solid arrows) of the right petrous apex as viewed on different axial MRI sequences. (C) The lesion is hypointense on diffusion trace image.


Close-up view. Sagittal.


Axial of the region of Dorello's canal. The lesion is seen to enter Dorello's canal (white arrows) and compress the right sixth nerve.


Axial view of head CTs taken in. 2011.


2016. A small lesion (arrow) causing mild bony abnormality of right petrous apex is visible on 2011. Both petrous apices are highly pneumatized. The lesion progressively erodes the right petrous apex over 5 years.


Postoperative MRI images. T2-weighted axial.


Postoperative MRI images. T1-weighted axial images after injection of gadolinium. Show a notable regression of the right petrous apex lesion (white arrows). The content of the lesion is still heterogeneous. There is slight peripheral enhancement.


CT Chest: Bilateral effusion, left more than right. Note diffuse soft tissue edema in the anterior aspect of left hemithorax.


CT Neck: Diffuse soft tissue edema and multiple enlarged lymph nodes on the left side. The jugular veins are shown by the thick arrows.


FNAC of cervical lymph node: Smear shows cluster of cells with irregular nucleus exhibiting moderate pleomorphism and moderate amount of vacuolated cytoplasm - consistent with metastatic adenocarcinoma.


Gastroendoscopy: Ulcer in the gastric antrum.


Gastric ulcer biopsy: Section shows fragments of gastric mucosa. The lamina propria shows moderate increase in the inflammatory cell content composed of lymphocytes and plasma cells. One of the fragments show sheets and clusters of neoplastic cells with high N:C, peripherally pushed pleomorphic and hyperchromatic nucleus with abundant eosinophilic granular cytoplasm. These cells are infiltrating the muscularis mucosa. Stomach biopsy consistent with moderately differentiated adenocarcinoma with signet ring cells.


Pedigree. The patient and his fourth brother showed heterozygous mutations in exon12 encoding PAH c.1068C>A and c.740G>T in WES, while the youngest brother was a carrier with only c.1068C>A. Other family members were roughly identified by positive clinical manifestations. No consanguineous marriage in this family.


Urine organic acid spectrum showed that phenylacetic acid, phenyllactic acid and Phenylpyruvate increased, suggesting hyperphenylalanine; 2.4-hydroxyphenyllactic acid increased, which may be secondary to liver function damage.


Pedigree chart of the family with FMD1. The asterisk indicates people tested for the FLNA pathogenic variant. The affected members are indicated by gray shading. Symbols divided into halves indicate heterozygous carriers of FMD1. The arrow indicates the proband.


Reverse DNA sequencing chromatogram of the patients and his family members.


Preoperative. Axial.


Sagittal T1-weighted contrast enhanced magnetic resonance imaging demonstrating a 2.5 x 3.7 x 2.5 cm heterogeneously enhancing cystic mass centered within the cerebellar vermis causing marked mass effect on the fourth ventricle.


Preoperative. Coronal.


Axial T1-weighted contrast enhanced magnetic resonance imaging demonstrating an 8 x 6 x 4 mm satellite lesion within the right superior cerebellar peduncle.


Bulky condyloma acuminata in the genital area.


Cervical spinal cord, cat. (A) Transversal section of formalin-fixed spinal cord with leptomeningeal pale, gray-beige mass (dashed line: junction tumor-spinal medulla).


Cervical spinal cord, cat. (B) Diffuse, basophilic cell infiltrates and edema within leptomeninges with compression of spinal cord. Spinal nerves (asterisks) are omitted by the tumor. HE stain.


Cervical spinal cord, cat. (C) Neoplastic infiltrates with junction to spinal medulla (bottom right) and necrosis (asterisk), HE stain. Scale bar: 50 mum.


Cervical spinal cord, cat. (D) High mitotic activity within the neoplasm (arrows), HE stain. Scale bar: 20 mum.


Spinal cord, cat. Tumor cells are diffusely immunopositive for OLIG2.


Spinal cord, cat. , doublecortin.


Spinal cord, cat.MAP2.


Spinal cord, cat. The majority of tumor cells stained positive with synaptophysin (D).


Spinal cord, cat. Positive vimentin reaction is mainly restricted to tumor:associated vasculature (E).


Spinal cord, cat. High proliferation activity was demonstrated with positive nuclear staining for Ki-67 (F). Scale bars: 50 mum.


Magnetic resonance imaging (MRI) revealed a 3 x 2.6-cm soft tissue mass in the anterior abdominal wall.


Peripheral smear showing marked eosinophilia.


Chest radiograph showing increased bronchovascular markings with right hilar prominence.


Sagittal.


Axial T2-weighted. Images demonstrated an intradural extramedullary cervical spine mass, displacing the spinal cord anteriorly and to the left.


Axial T1 precontrast.


Axial T1 postcontrast images. Demonstrate heterogeneous enhancement of the mass, with a convex border abutting the spinal cord.


Sagittal T2-weighted.


T1 FS postcontrast. Images of the cervical spine demonstrate no residual or recurrent tumor 7 months postoperatively.


Pretreatment radiological imaging. . Notes: (A) Coronal view of enhanced CT. Opacification of the left maxillary sinus, ethmoid sinus, and sphenoid sinus is revealed.


Pretreatment radiological imaging. (B) Axial view of enhanced CT. The posterolateral wall of the maxillary sinus is invaded and destroyed.


Pretreatment radiological imaging. (C) Coronal view of T2-weighted imaging. The tumor expands from the maxillary sinus to the common nasal meatus. The ethmoid sinus and nasofrontal duct are filled with secondary sinusitis.


Pretreatment radiological imaging. (D) Axial view of Gd enhanced T1-weighted imaging. The tumor extends posterior-laterally, invading the medial and lateral pterygoid muscles. . Abbreviation: CT, computed tomography.


(A and B) Hematoxylin-eosin stain revealed spindle cells and small round cells with ovoid hyperchromatic nuclei arranged in cellular sheets.


(A and B) Hematoxylin-eosin stain revealed spindle cells and small round cells with ovoid hyperchromatic nuclei arranged in cellular sheets. (Original magnification A: x100, B: x400.


Immunohistochemically, the tumor cells were diffusely reactive for TLE1.


Magnetic resonance imaging after three courses of ifosfamide and pirarubicin. . Notes: No residual tumor can be identified. (A) Coronal view of Gd-enhanced T1-weighted imaging.


Magnetic resonance imaging after three courses of ifosfamide and pirarubicin. . Notes: No residual tumor can be identified. (B) Axial view of T2-weighted imaging.


The edge of a tumorous mass is found at the lower margin of the upper eyelid. There is no loss of eye lashes (a). On lid eversion, there is a yellowish multilobulated papillary tumor behind the lid.


The edge of a tumorous mass is found at the lower margin of the upper eyelid. The tumor is pedunculated from the tarsal palpebral conjunctiva (b).


A histopathologic section (hematoxylin-eosin, original magnification x200) from the biopsied specimen shows that the tumor consists of proliferative atypical vacuolated cells with slight basophilic cytoplasms and atypical nuclei associated with hyperchromatic nucleoli (a).


A histopathologic section (hematoxylin-eosin, original magnification x12.5) from the resected specimen shows that the tumor demonstrated papillary and pedunculated exophytic growth from the palpebral conjunctiva (b).


Greater magnification of the black square (hematoxylin-eosin, original magnification x100) reveals slight invasion into the tarsus (c). T, tumor; TA, tarsus.


Intraoperative appearance of nodular lesion over the upper lip and excised lesion.


Histological picture of the lesion (hematoxylin and eosin stain).


Chest X-ray and chest computed tomography (CT) at admission. (A) Chest X-ray showing a large pleural effusion in the left hemithorax.


Chest X-ray and chest computed tomography (CT) at admission. Chest CT,. Showing a huge pleural mass with pleural effusion in the left hemithorax.


Chest X-ray and chest computed tomography (CT) at admission. Chest CT,. A pulmonary nodule in the left upper lobe. Arrow).


Chest X-ray and chest computed tomography (CT) at admission. Chest CT,. A right pleural nodule. Arrow).


Positron emission tomography-computed tomography (PET-CT) scans. (A) A focal hypermetabolic nodular lesion is seen in the left upper lobe.


Positron emission tomography-computed tomography (PET-CT) scans. (B) A large pleural effusion with heterogeneous minimal-to-mild hypermetabolic activity is seen in the left lower lobe.


Positron emission tomography-computed tomography (PET-CT) scans. (C) Minimal hypermetabolic nodular lesion is seen in right lower pleural surface (arrow). No other hyperrmetabolic lesions suggesting a primary tumor are seen in this PET-CT image.


Light microscopy features of the resected pleural and renal tumors. The renal tumor was resected in another hospital 20 yr previously and diagnosed as a low-grade clear cell renal cell carcinoma. (A) The pleural tumor shows an organoid or nesting arrangement of cells with a pseudoalveolar pattern. The tumor cells are separated by fibrovascular septa and delicate capillary-sized vascular channels (H&E, x 200).


Light microscopy features of the resected pleural and renal tumors. The renal tumor was resected in another hospital 20 yr previously and diagnosed as a low-grade clear cell renal cell carcinoma.


Light microscopy features of the resected pleural and renal tumors. The renal tumor was resected in another hospital 20 yr previously and diagnosed as a low-grade clear cell renal cell carcinoma. (C) Immunohistochemistry of the pleural tumor. The nuclei are positive for TFE3 (TFE3, x 400).


Light microscopy features of the resected pleural and renal tumors. The renal tumor was resected in another hospital 20 yr previously and diagnosed as a low-grade clear cell renal cell carcinoma. The cytoplasms of the tumor cells are D-PAS positive PAS, x 200). (D) The tumor cells of right renal mass have clear cytoplasms, distinct cell borders, and small nuclei. The cells are arranged in small solid nests (H&E, x 200).


The left lower extremity.


The right lower extremity. Preoperatively demonstrating gouty tophi with overlying ulceration to the left lower extremity.


Plain radiograph of the left lateral knee preoperatively demonstrating a soft tissue mass with calcium precipitation overlying the extensor mechanism.


Radiographic image of the right lower knee preoperatively demonstrating a soft tissue mass with calcium precipitation overlying the extensor mechanism.


Axial T2-weighted magnetic resonance imaging of the left knee preoperatively revealing an enhancing and heterogeneous soft tissue mass overlying the extensor mechanism.


Histological evidence of biopsy showing gouty tophus.


The left knee postoperatively demonstrating wound closure with a Jackson-Pratt drain.


The right knee gouty tophi preoperatively.


During perioperative exposure.


Post-operative images of the right.


Left. Knee.


Noncontrast axial computed tomography at bone algorithm showing a tumoral mass occupying the posterior part of the right mandible.


Three-dimensional tomographic reconstruction showing cortical expansion and fenestration.


Intraoral image of the tumor during surgery.


The image of tumor cavity in the posterior region of the mandible after tumor was removed.


Patient's orthopantograph 6 months after the surgery.


After the surgery fixed prosthesis was made on the surgical side.


Photograph of bilateral lower extremities showing gross right limb enlargement, hypertrophy, disfigurement and skin ulceration.


Photograph of the back of the trunk showing multiple "cafe au lait" spots of variable sizes.


Slit lamp examination of the right eye showing multiple (five) pigmented iris hamartomas (Lisch nodules).


Gray scale ultrasonography of the right lower limb shows multilobulated tortuous entanglement of tumors, oriented along the long axis of the nerve on longitudinal section, and "target sign" on transverse scan, evident as echogenic center and hypoechoic periphery.


Plain computed tomography (bone window) demonstrates a large periosteal defect giving way to the infiltrating mass lesion, periosteal/ endosteal thickening (axial and coronal image-arrows) and cortical erosions at talocalcaneal joint (sagittal image-arrows).


Plain computed tomography (soft tissue window) shows thick wavy cords of low attenuated masses traversing the soft tissue and giving a reticular-network appearance to the right leg.


Histopathology of the resected mass reveals a large number of irregular infiltrative spindle cells, several nerve segments of varying length embedded in subcutaneous fat, many mast cells and myxoid stroma - finding consistent with diffuse plexiform neurofibromatosis.


Blackish brown proliferative growth on edentulous maxillary arch, and ,palate.


Small blackish macules on the soft palate.


Contrast enhanced computed tomography showing (c) a large infiltrating homogenous mass on right anterior alveolus.


(d) heterogenous enlarged submandibular lymph nodes.


(a and b) Brownish black, irregular growth extending from 13 to 27 on both buccal and lingual aspects involving ridge area.


Contrast enhanced computed tomography showing. A homogeneous mass with the erosion of left alveolar arch.


Extension into the left lateral margin of hard palate.


(a) Well circumscribed ovoid growth on maxillary anterior region.


(b) Contrast enhanced computed tomography showing heterogenous soft tissue mass perforating the anterior hard palate.


(c and d) 18F-fluorodeoxyglucose positron emission tomography-computed tomography scan showing metastatic deposits in lymph nodes (cervical, supraclavicular, mediastinal, and abdominal), liver, lung, and brain. Inset shows multiple deposits in the brain.


Lymph nodes from the patient a; The patient has normal lymph node architecture.


Lymph nodes from the patient b; Showing reduced numbers of plasma cells in the germinal centre in the patient.


Normal control Plasma cells staining with CD138 are shown in brown. C; The plasma cells are in the interfollicular areas in the control. CVID is typically associated with the absence of plasma cells and poorly formed germinal centres. The patient was not on immunosuppression at the time of the lymph node excision.


MRI of the cauda equina. Enhancing lesions of the nerve roots are seen consistent with granulomatous inflammation. A biopsy was not possible, given the location.


IgG levels during immunosuppression. In mid-2018 the IgG normalised briefly between resolution of the cauda equine syndrome and treatment of renal disease. Cauda Cauda equine syndrome, Evans Evans syndrome, ITP immune thrombocytopenia, IVIG intravenous immunoglobulin, IS immunosuppression, Renal renal impairment.


Clinical. (a) A 55-year-old female with swelling in the right parasymphysis region. (b) Swelling extending from 43 to 46 region. H/O extraction of 44 and 45 2 years ago. (c) Ill-defined radiolucency extending from 43 to mesial aspect of 47 causing thinning of lower border of the mandible.


A dark-red nodule with extended purpura on the right femur with prominent lymph edema (a).


Irregularly anastomosing vascular channels lined by single layers of enlarged, atypical endothelial cells that existed between the collagen bundles (H&E staining) (b).


Immunohistochemical staining for case 1: IL-23.


Multiple dark-red nodules with extended purpura on the left femur with prominent lymph edema (a).


Irregularly anastomosing vascular channels lined by single layers of enlarged, atypical endothelial cells that existed between collagen bundles with prominent apoptotic cells (b).


Immunohistochemical staining for case 2: IL-23.


Mucosal colored swelling of approximately 3 cm in diameter on the lingual aspect of mandible on the right side, extending from 32 to 44.


Swelling of approximately 3 cm in diameter on the lingual aspect of mandible on the right side, extending from 32 to 44.


IOPA shows a diffuse radiolucent area with scattered flecks of radiopacities at the apical region of 41-43.


Odontogenic epithelial cells arranged in the form of follicles and stellate reticulum like cells in the center which are surrounded by ectomesenchymal cells (4x).


tall columnar ameloblast like cells on the periphery with stellate reticulum like cells at the center and surrounded by condensed mesenchymal cells (10x).


Neoplastic odontogenic epithelium in the form of large follicles (10x).


Tall columnar ameloblasts like cells showing nuclear palisading, reversal of polarity and stellate reticulum like cells (40x).


Dense ectomesenchymal cells present in the connective tissue stroma (40x).


large masses of dysplastic dentin arranged in a haphazard pattern. (10x).


Irregular masses of dysplastic dentin, areas of calcification and stromal connective tissue(10x).


Sagittal.


Coronal. Results from gadolinium-enhanced T1-weighted magnetic resonance imaging.


Gross total resection of the tumor was confirmed based on the sagital.


Coronal.


Axial results. From the postoperative computed tomography.


Four-month follow-up axial.


Sagittal results. From T1-weighted magnetic resonance imaging revealed a hyperintense sellar mass, which was compatible with an autologous fat graft that was used to prevent nasal fistula.


Findings from T1-weighted gadolinium-enhanced magnetic resonance imaging after 4 months.


18 months.


Clinical photograph showing excessive hair growth of eyelashes.


Slit lamp examination demonstrating the presence of Kayser-Fleischer rings, seen as a dark brown arc at the corneal limbus.


Lymph node aspirate with scattered epithelioid cell granuloma (Giemsa, x20). Inset: Numerous intracellular as well as extracellular Leishman-Donovan bodies (Giemsa, x40).


(a, b) Radiographs of the cervical and thoracic spine demonstrating fused vertebral bodies from C2 down to T6.


No instability was documented on flexion-extension studies, (c) Significant kyphosis at the higher thoracic spine.


(a-d) Pre-operative cranio-cervico-thoracic CT scan showing a mass in the posterior fossa behind the fourth ventricle and below the cerebellum extending down behind the spinal cord to the posterior thoracic vertebra to T1. In the posterior fossa, it obstructed the foramen of Magendie, thus resulting in secondary obstructive hydrocephalus. In addition, fusion of the cervical and thoracic vertebral bodies from C2 to T6, involving the anterior spinal column and posterior elements is seen.


(a-c) CT-angiography showing the vertebral arteries located anterior to the lesion in addition to a low-lying venous confluence.


Pre-operative magnetic resonance imaging, T1WI sagittal.


T2WI sagittal.


Stir axial. Sections documenting an expansive midline lesion extending from the cranio-cervical junction down to T1. The dermal sinus tract, extending from the dermis to the suboccipital bone below the occipital protuberance is identified on T1WI and T2WI (arrow).


Intraoperative image of the dermoid cyst.


Postoperative magnetic resonance imaging, T1WI sagittal.


T2WI sagittal. Sections and T1WI axial.


Postoperative plain lateral cervicothoracic radiograph demonstrating no new-onset deformity at 6-year follow-up.


Postoperative magnetic resonance imaging, T2WI sagittal.


T2WI axial.


T1WI post-gadolinium axial sections demonstrating no recurrence at 6 years after surgery. Demonstrating no recurrence at 6-year follow-up.


Left-sided huge swelling of the neck (note the tracheal shift to the right side due to mass effect).


Tongue deviation to the left (affected) side.


H and E-stained sections from the mass. Sections showing biphasic tumour formed of Antoni A areas (black arrows), with Verocay bodies (blue arrows), and Antoni B areas (white arrow).


A; Histological microphotograph showing a densely pigmented mass involving the iris, ciliary body, trabecular meshwork, and Schlemm's canal. Hematoxylin and eosin.


40x. B; A bleached preparation revealing large tumor cells with central and paracentral nuclei. The tumor cells show a low nuclear-to-cytoplasmic ratio without mitosis (bleached preparation. Hematoxylin and eosin. 200x).


C; An ultrastructural microphotograph showing melanin-containing tumor cells (white arrow) and trabecular meshwork cells (black arrow) in the trabecular meshwork. The melanin granules are not phagocytosed by the trabecular cells (electron micrograph, 2,000x).


Exposed patella with 4 x 4 cm skin defect and visible patella fixation sutures.


Harvesting gastrocnemius muscle fascia.


Patella tendon reinforced with the remaining fascia.


Twelve months after the surgery.