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ERROR: type should be string, got "https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTE3NzA2OS1vdmVydmlldw==\nDrugs & Diseases > Neurology\nNeonatal Seizures\nAuthor: Raj D Sheth, MD; Chief Editor: Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP more...\nSections Neonatal Seizures\nSeizure Medications\nAnticonvulsants, Other\nVitamins, Water-Soluble\nThe most prominent feature of neurologic dysfunction in the neonatal period is the occurrence of seizures. Determining the underlying etiology for neonatal seizures is critical. Etiology determines prognosis and outcome and guides therapeutic strategies. [1] (See Etiology, Prognosis, Treatment, and Medication.)\nThe neonatal period is limited to the first 28 days of life in a term infant. For premature infants, this term usually is applied until gestational age 44 weeks; ie, the age of the infant from conception to 44 weeks (ie, 4 wk after term).\nSeizure characteristics\nMost neonatal seizures occur over only a few days, and fewer than half of affected infants develop seizures later in life. Such neonatal seizures can be considered acute reactive (acute symptomatic), and therefore the term neonatal epilepsy is not used to describe neonatal seizures. [2]\nSeizures in neonates are relatively common, with variable clinical manifestations. Their presence is often the first sign of neurologic dysfunction, and they are powerful predictors of long-term cognitive and developmental impairment. (See Prognosis.)\nMost seizures in the neonate are focal, although generalized seizures have been described in rare instances.\nWhat have been termed \"subtle seizures\" are more common in full-term than in premature infants. Video electroencephalogram (EEG) studies have demonstrated that most subtle seizures are not associated with electrographic seizures. Examples of subtle seizures include chewing, pedaling, or ocular movements, these movements are thought not be epileptic in nature and more commonly are an epi-phenomena of severe encephalopathy. [3]\nNeonatal seizure classification\nThese movements most commonly are associated with electrographic seizures. They often involve 1 extremity or 1 side of the body. The rhythm of the clonic movements is usually slow, at 1-3 movements per second.\nThese may involve 1 extremity or the whole body. Focal tonic seizures involving 1 extremity often are associated with electrographic seizures.\nGeneralized tonic seizures often manifest with tonic extension of the upper and lower limbs and also may involve the axial musculature in an opisthotonic fashion. Generalized tonic seizures mimic decorticate posturing; the majority are not associated with electrographic seizures.\nThese may occur focally in 1 extremity or in several body parts (in which case they are described as multifocal myoclonic seizures).\nFocal and multifocal myoclonic seizures typically are not associated with electrographic correlates. These movements are thought to be non-epileptic in nature and a reflection of severe encephalopathy.\nThe biochemical effects of neonatal seizures include derangements of energy metabolism. Energy-dependent ion pumps are compromised, and adenosine diphosphate (ADP) levels rise. The rise in ADP stimulates glycolysis with the ultimate increase in pyruvate, which accumulates as a result of compromised mitochondrial function.\nFor patient education information, see the Brain and Nervous System Center, as well as Seizures in Children and Seizures Emergencies.\nSeizures occur when a large group of neurons undergo excessive, synchronized depolarization. Depolarization can result from excessive excitatory amino acid release (eg, glutamate) or deficient inhibitory neurotransmitter (eg, gamma amino butyric acid [GABA]).\nHypoxic-ischemic encephalopathy\nAnother potential cause is disruption of adenosine triphosphate (ATP) ̶ dependent resting membrane potentials, which cause sodium to flow into the neuron and potassium to flow out of the neuron. Hypoxic-ischemic encephalopathy disrupts the ATP-dependent sodium-potassium pump and appears to cause excessive depolarization. It is an important cause of neonatal seizures. [1, 4]\nSeizures resulting from hypoxic-ischemic encephalopathy may be seen in term and premature infants. They frequently present within the first 72 hours of life. Seizures may include subtle, clonic, or generalized seizures.\nIntracranial hemorrhage occurs more frequently in premature than in term infants. Distinguishing infants with pure hypoxic-ischemic encephalopathy from those with intracranial hemorrhage often is difficult.\nSubarachnoid hemorrhage is more common in term infants. This type of hemorrhage occurs frequently and is not clinically significant. Typically, infants with subarachnoid hemorrhage appear remarkably well.\nGerminal matrix-intraventricular hemorrhage is seen more frequently in premature than in term infants, particularly in infants born prior to 34 weeks' gestation. Subtle seizures are seen frequently with this type of hemorrhage.\nSubdural hemorrhage is seen in association with cerebral contusion. It is more common in term infants.\nMetabolic disturbances include hypoglycemia, hypocalcemia, and hypomagnesemia. Less frequent metabolic disorders, such as inborn errors of metabolism, are seen more commonly in infants who are older than 72 hours. Typically, they may be seen after the infant starts feeding.\n\"Early-onset epileptic encephalopathy\" refers to a syndrome in which seizures are refractory to medications and severe cognitive/developmental issues are present. In those patients in whom structural and metabolic causes have been ruled out, genetic mutations are increasingly recognized. These mutations occur in genes that code for ion channel subunits (such as SCN1A, SCN8A, KCNT1) and other nueronal proteins and enzymes (such as CDKL5, STXBP1). [5]\nIntracranial infections\nIntracranial infections (which should be ruled out vigorously) that are important causes of neonatal seizures include meningitis, encephalitis (including herpes encephalitis), toxoplasmosis, and cytomegalovirus (CMV) infections. The common bacterial pathogens include Escherichia coli and Streptococcus pneumoniae.\nMalformation syndromes\nWhile most cerebral malformations present with seizures at a later age, major malformation syndromes are important to consider. Lissencephaly, pachygyria, polymicrogyria, and linear sebaceous nevus syndrome can present with seizures in the neonatal period.\nBenign neonatal seizures\nBenign neonatal seizure syndromes can be characterized by familial or idiopathic seizures. Benign familial neonatal seizures typically occur in the first 48-72 hours of life; the seizures disappear by age 2-6 months. A family history of seizures is usual. Development is typically normal in these infants.\nBenign idiopathic neonatal seizures typically present at day 5 of life (ie, fifth day fits), with the vast majority presenting between days 4 and 6 of life. Seizures are often multifocal. Cerebrospinal fluid (CSF) analysis is usually unremarkable.\nThe incidence of neonatal seizures in the United States has not been clearly established, although an estimated frequency of 80-120 cases per 100,000 neonates per year has been suggested. The incidence of seizures is higher in the neonatal period (ie, the first 4 wk after birth) than at any other time of life. [6]\nAge-related demographics\nNeonatal seizures by definition occur within the first 4 weeks of life in a full-term infant and up to 44 weeks from conception for premature infants. Seizures are most frequent during the first 10 days of life.\nPrognosis is determined by the etiology of the neonatal seizures. If the EEG background is normal, the prognosis is excellent for seizures to resolve; normal development is likely. [7, 8]\nSevere EEG background abnormalities indicate poor prognosis; such patients frequently have cerebral palsy and epilepsy. The presence of spikes on EEG is associated with a 30% risk of developing future epilepsy.\nThe prognosis following neonatal seizures that result from isolated subarachnoid hemorrhage is excellent, with 90% of children not having residual neurologic deficits.\nPisani et al devised a scoring system for early prognostic assessment after neonatal seizures. Analysis of 106 newborns with neonatal seizures who were followed prospectively to 24 months' postconceptional age identified 6 independent risk factors for adverse outcome: (1) birth weight, (2) Apgar score at 1 minute, (3) neurologic examination at seizure onset, (4) cerebral ultrasonogram, (5) efficacy of anticonvulsant therapy, and (6) presence of neonatal status epilepticus.\nEach variable was scored from 0 to 3 to represent the range from normal to severely abnormal; these were then added together to produce a total composite score, ranging from 0 to 12. A cutoff score of 4 or higher provided the greatest sensitivity and specificity for prediction of adverse neurologic outcome. [9]\nMorbidity and mortality\nNeonatal seizures are a risk factor that markedly increases rates of long-term morbidity and neonatal mortality. The presence of neonatal seizures is the best predictor of long-term physical and cognitive deficits. Complications of neonatal seizures may include the following:\nCerebral palsy/spasticity\nCerebral atrophy/Hydrocephalus ex-vacuo\nFeeding difficulties\nVolpe JJ. Hypoxic-Ischemic Encephalopathy: Biochemical and Physiological Aspects. Neurology of the Newborn. 4th ed. Philadelphia: WB Saunders; 2000. 217-276.\nLombroso CT. Neonatal seizures: gaps between the laboratory and the clinic. Epilepsia. 2007. 48 Suppl 2:83-106. [Medline].\nSheth RD. Electroencephalogram confirmatory rate in neonatal seizures. Pediatr Neurol. 1999 Jan. 20(1):27-30. [Medline].\nSilverstein FS, Jensen FE. Neonatal seizures. Ann Neurol. 2007 Aug. 62(2):112-20. [Medline].\nHwang SK, Kwon S. Early-onset epileptic encephalopathies and the diagnostic approach to underlying causes. Korean J Pediatr. 2015 Nov. 58 (11):407-14. [Medline].\nSheth RD, Hobbs GR, Mullett M. Neonatal seizures: incidence, onset, and etiology by gestational age. J Perinatol. 1999 Jan. 19(1):40-3. [Medline].\nSheth RD. Frequency of neurologic disorders in the neonatal intensive care unit. J Child Neurol. 1998 Sep. 13(9):424-8. [Medline].\nSheth RD, Bodensteiner JB. Delayed postanoxic encephalopathy: possible role for apoptosis. J Child Neurol. 1998 Jul. 13(7):347-8. [Medline].\nPisani F, Sisti L, Seri S. A scoring system for early prognostic assessment after neonatal seizures. Pediatrics. 2009 Oct. 124(4):e580-7. [Medline].\nVigevano F. Benign familial infantile seizures. Brain Dev. 2005 Apr. 27(3):172-7. [Medline].\nSheth RD, Buckley DJ, Gutierrez AR, et al. Midazolam in the treatment of refractory neonatal seizures. Clin Neuropharmacol. 1996 Apr. 19(2):165-70. [Medline].\nCherian PJ, Deburchgraeve W, Swarte RM, De Vos M, Govaert P, Van Huffel S, et al. Validation of a new automated neonatal seizure detection system: a clinician's perspective. Clin Neurophysiol. 2011 Aug. 122(8):1490-9. [Medline].\nSheth RD. Electroencephalogram in developmental delay: specific electroclinical syndromes. Semin Pediatr Neurol. 1998 Mar. 5(1):45-51. [Medline].\nGlass HC, Shellhaas RA, Wusthoff CJ, Chang T, Abend NS, Chu CJ, et al. Contemporary Profile of Seizures in Neonates: A Prospective Cohort Study. J Pediatr. 2016 Jul. 174:98-103.e1. [Medline].\nShah DK, de Vries LS, Hellström-Westas L, Toet MC, Inder TE. Amplitude-integrated electroencephalography in the newborn: a valuable tool. Pediatrics. 2008 Oct. 122(4):863-5. [Medline].\nNatalucci G, Leuchter RH, Ulrich Bucher H, Latal B, Koller B, Hüppi PS, et al. Functional brain maturation assessed during early life correlates with anatomical brain maturation at term equivalent age in preterm infants. Pediatr Res. 2013 Apr 5. [Medline].\nPeliowski-Davidovich A,. Hypothermia for newborns with hypoxic ischemic encephalopathy. Paediatr Child Health. 2012 Jan. 17(1):41-6. [Medline].\nScher MS, Trucco GS, Beggarly ME, et al. Neonates with electrically confirmed seizures and possible placental associations. Pediatr Neurol. 1998 Jul. 19(1):37-41. [Medline].\nSankar R, Painter MJ. Neonatal seizures: after all these years we still love what doesn't work. Neurology. 2005 Mar 8. 64(5):776-7. [Medline].\nPainter MJ, Scher MS, Stein AD, et al. Phenobarbital compared with phenytoin for the treatment of neonatal seizures. N Engl J Med. 1999 Aug 12. 341(7):485-9. [Medline].\nKhan O, Cipriani C, Wright C, Crisp E, Kirmani B. Role of intravenous levetiracetam for acute seizure management in preterm neonates. Pediatr Neurol. 2013 Nov. 49 (5):340-3. [Medline].\nOnset of neonatal seizure demonstrating a focal onset in the right frontal (FP4) region. At this point, the child had head and eye deviation to the left.\nTwenty seconds into a seizure that had focal onset in the right frontal (FP4) region, the seizure shows a rhythmic buildup of activity in the right frontocentral region.\nThis seizure had focal onset in the right frontal (FP4) region and subsequent buildup of activity in the right frontocentral region. As the seizure evolves, the electroencephalogram shows diffuse involvement of both cerebral hemispheres.\nRaj D Sheth, MD Chief, Division of Pediatric Neurology, Nemours Children's Clinic; Professor of Neurology, Mayo Clinic Alix School of Medicine; Professor of Pediatrics, University of Florida College of Medicine\nRaj D Sheth, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Neurological Association, Child Neurology Society\nDisclosure: AAN reviewer, ACNS Ed board, Infantile spasms consultant for: AAN; ACNS; Mackilrodt.\nStephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP Chief of Pediatric Neurology, Professor of Pediatrics, Neurology, Neurosurgery, and Psychiatry, Epileptologist, Medical Director, Tulane Center for Autism and Related Disorders, Co-Director, Developmental Neurogenetics Center, Tulane University School of Medicine\nStephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Medical Association, Association of Military Surgeons of the US, Child Neurology Society, Southern Pediatric Neurology Society\nDisclosure: Serve(d) as a speaker or a member of a speakers bureau for: Biomarin; Supernus<br/>Received income in an amount equal to or greater than $250 from: Biomarin; Supernus, Sunovion; American Board of Pediatrics.\nRobert Stanley Rust Jr, MD, MA Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-Director of FE Dreifuss Child Neurology and Epilepsy Clinics, Director, Child Neurology, University of Virginia; Chair-Elect, Child Neurology Section, American Academy of Neurology\nRobert Stanley Rust Jr, MD, MA is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Headache Society, American Neurological Association, Child Neurology Society, International Child Neurology Association, and Society for Pediatric Research\nFrancisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference\nDisclosure: Medscape Salary Employment\nencoded search term (Neonatal Seizures) and Neonatal Seizures\nBenign Childhood Epilepsy\nWomen's Health and Epilepsy\nEEG in Common Epilepsy Syndromes\nOutcome of Epilepsy Surgery\nIdentification of Potential Epilepsy Surgery Candidates\nPosttraumatic Epilepsy\nSeizure Disorders in Pregnancy\nMajor Financial Impact, Disability Tied to Post-TBI Epilepsy\nFDA Clears First Nasal Spray for Cluster Seizures in Kids 6 Years and Older\nDiet and Mental Health: The Evidence to Date\nThe 10 Graphic Novels That Made Graphic Medicine\nAccording to Neurologists\nFDA Warns of 'Serious' Respiratory Problems With Gabapentin\nBedtime Dosing of Hypertension Meds Reduces CV Events\nNovel Over-the-Counter Device May Offer Long-Lasting Pain Relief\n'Low Value' Care? More Controversy for Statins in Primary Prevention\nAspirin Key to Expanding Access to Effective Migraine Treatment?\nNews Major Financial Impact, Disability Tied to Post-TBI Epilepsy\nDiseases & Conditions Posttraumatic Epilepsy\nNews AI Sheds New Light on Suicidality in Epilepsy\nDiseases & Conditions Psychiatric Disorders Associated With Epilepsy"

ERROR: type should be string, got "https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMzY5MzY0LW92ZXJ2aWV3\nFibrolamellar Hepatocellular Carcinoma Imaging\nAuthor: W Ross Stevens, MD; Chief Editor: John Karani, MBBS, FRCR more...\nSections Fibrolamellar Hepatocellular Carcinoma Imaging\nFibrolamellar hepatocellular carcinoma, or fibrolamellar carcinoma, is a rare primary malignant neoplasm of the liver that constitutes 0.8-16% of all hepatocellular malignancies. [1, 2] It typically affects persons between 5 and 35 years, but there appears to be 2 peak incidences, at age 10-30 years and at 70-79 years. [3, 4, 5] Fibrolamellar carcinoma has distinctive clinical, histologic, [6, 7] and radiographic features that distinguish it from the relatively more common hepatocellular carcinoma (HCC). It often presents with vague abdominal pain, nausea, abdominal fullness, malaise, and weight loss. [8] Fibrolamellar carcinoma occurs in a younger population than does HCC, and it is typically not associated with underlying liver disease or elevated serum levels of alpha-fetoprotein tumor markers. [9] Also, fibrolamellar carcinoma may have a slightly better prognosis than HCC. [10, 11]\n(See the images of fibrolamellar hepatocellular carcinoma below.)\nContrast-enhanced computed tomography (CT) scan of fibrolamellar carcinoma demonstrates a large, heterogeneously enhancing mass in the right lobe of the liver, with a hypoattenuating central scar and punctate, central calcification.\nDigital subtraction arteriogram (obtained by injection of the celiac axis) demonstrates fibrolamellar carcinoma; the carcinoma appears as an area of hypervascularity and tumor blush in the inferior aspect of the liver.\nPerhaps because of the younger ages of the patients and the lack of coexisting cirrhosis, patients with fibrolamellar carcinoma are often treated aggressively. Resection of large tumor masses, of metastatic disease, and even of recurrent disease can extend patient survival.\nPreferred examination\nAbdominal computed tomography (CT) scanning is the preferred examination for the detection, diagnosis, staging, and postoperative follow-up evaluation of fibrolamellar carcinoma. For the best evaluation of the liver and liver tumors with CT scanning, the intravenous administration of iodinated contrast material is necessary. Therefore, the use of CT scanning is limited in patients who cannot have iodinated contrast material because of an allergy or renal insufficiency. In these patients, MRI is preferred. In addition, CT scanning uses ionizing radiation; therefore, it can be relatively contraindicated in children or in women of childbearing age, in whom radiation doses should be limited as much as possible. [10, 12, 13] On contrast-enhanced CT, fibrolamellar HCC typically presents as a large (7-20 cm), heterogeneous, well-defined mass with a lobulated outline. On unenhanced CT, fibrolamellar HCC is most often hypoattenuating with calcifications (40–68%) and a central stellate scar (65–75%). [8, 14, 15, 16]\nMagnetic resonance imaging (MRI) of the liver can be useful in detecting and characterizing primary tumors, and MRI may be slightly more sensitive than CT scanning in detecting multiple intrahepatic recurrent lesions. However, MRI is less sensitive than CT scanning in the detection of extrahepatic disease. Tumors are usually hypointense on T1-weighted images and hyperintense on T2-weighted images, with a fibrous central scar that remains hypointense on both T1- and T2-weighted images. On gadolinium-enhanced MRI, fibrolamellar HCC is characterized by marked heterogeneous enhancement on the arterial phase that washes out and leaves an isointense or hypointense lesion on the portal venous phase. [17, 18, 19, 8, 15, 16]\nThe imaging time with MRI examinations tends to be longer than with CT scanning or ultrasonography. In patients who are unwilling or unable to remain still during the imaging period, poor-quality images can result because of motion artifact; these artifacts can limit the usefulness of the MRI examination. Some patients may become claustrophobic while they are in the MRI machine; patients may require sedation, or they may not be able to complete the examination. Because of the high magnetic field strength, MRI is contraindicated in patients with a cardiac pacemaker or some internal metallic object, such as an aneurysm clip or metal shrapnel or filings in a critical location. [17, 12, 10, 18, 19]\nOn ultrasound, fibrolamellar hepatocellular carcinoma is characterized as a well-defined mass that has heterogenous echogenicity. Ultrasonography has been used in the surveillance of the progression of known intrahepatic lesions. Percutaneous biopsy with CT scanning or ultrasonographic guidance may be necessary for a definitive preoperative diagnosis of fibrolamellar carcinoma. Ultrasonography is more operator dependent than CT scanning or MRI, and poor technique in scanning can limit its diagnostic usefulness. Ultrasonography may be limited by the patient's anatomy. Hepatic imaging may be difficult in obese patients or in patients whose liver is located high under the ribs. Overlying bowel gas may obscure portions of the liver in some patients. [8]\nRadionuclide sulfur-colloid scans or scans obtained with labeled red blood cells are occasionally useful in the differentiation of fibrolamellar carcinomas from other types of hepatic tumors.\nPlain radiographs of the abdomen may demonstrate nonspecific secondary findings related to hepatomegaly or to the abdominal mass in patients with fibrolamellar carcinoma. Findings may include an enlarged liver shadow and/or displacement of adjacent, gas-filled bowel loops. Calcifications in the tumor also may be visible on plain abdominal images.\nPlain radiographic findings in patients with fibrolamellar carcinoma are nonsensitive and nonspecific. These findings are not helpful in the detection or characterization of the tumors.\nAny cause of space-occupying abdominal masses or hepatomegaly can produce plain radiographic findings similar to those of fibrolamellar carcinoma, thereby causing a false-positive finding. Changes associated with fibrolamellar carcinoma may not produce any abnormality on plain radiographs, thereby causing a false-negative result.\nAbdominal CT scanning is the preferred imaging method for the diagnosis, staging, and follow-up surveillance of fibrolamellar carcinoma. CT scanning has high sensitivity in the detection of intrahepatic tumors and regional lymph node metastases. CT scanners provide the ability to scan the liver without enhancement and, during the arterial and portal venous phases, after the intravenous administration of iodinated contrast material. [10, 12, 14]\nOn nonenhanced scans, the primary fibrolamellar tumor typically appears as a large, solitary, hypoattenuating mass with well-circumscribed and lobulated margins. During the arterial-enhancing phase, the tumor is heterogeneously enhancing and becomes generally hyperattenuating with respect to the relatively less strongly enhancing surrounding liver. During the portal and delayed phases, the tumor remains enhancing and becomes more homogeneous in appearance, with its density more closely matching that of the liver as equilibrium is achieved.\nCentral scars (as seen in the image below) are present in 50-70% of fibrolamellar carcinomas and appear on CT scans as a central stellate hypoattenuating and hypoenhancing region in the mass. The scars may not be enhancing at all, or they may show mild enhancement on delayed-enhanced scans. Calcifications occur in 35-60% of fibrolamellar tumors, and they can be best identified as hyperattenuating foci on nonenhanced CT scans.\nMetastatic lymphadenopathy is present at the time of initial diagnosis of fibrolamellar carcinoma in 50-70% of patients, and the lymph nodes are frequent sites for recurrent disease after surgical resection of primary lesions. Lymph node metastases are found most often in the porta hepatis (as demonstrated in the image below), and they can have a CT scan appearance similar to that of intrahepatic lesions. Other distant metastases, such as those to the lungs, peritoneum, or more distant lymph nodes, also can be detected by using CT scanning.\nComputed tomography (CT) scan in a patient with fibrolamellar carcinoma shows metastatic lymphadenopathy in the porta hepatis–gastrohepatic region.\nAbdominal CT scanning is sensitive in detecting the primary intrahepatic tumors of fibrolamellar carcinoma and of regional lymph node metastases. When found in the appropriate clinical setting of a young patient without cirrhosis, the characteristic CT scan appearance of a large, lobulated, heterogeneously enhancing tumor with a central scar and calcification, often with local lymph node metastases, can provide a confident diagnosis of fibrolamellar carcinoma. Because most patients are treated with surgical resection, percutaneous biopsy may be useful in the preoperative confirmation of the diagnosis.\nIn an atypical clinical setting, fibrolamellar carcinomas may be confused with hepatocellular adenomas or HCC, which can have a similar appearance. HCC often presents as a single, large, well-circumscribed encapsulated hypoattenuating lesion on unenhanced CT, while other tumor features such as fat involvement, foci of hemorrhage, and necrotic areas are more common in noncirrhotic HCC. [20] .\nFocal nodular hyperplasia (FNH) can appear similar to fibrolamellar tumors, but its enhancement tends to be more homogeneous. Also, FNH has an enhancing scar, and it does not metastasize. [13]\nFibrolamellar carcinoma is typically identified on MRI scans as a large, well-defined, lobulated mass. On T1-weighted images, the tumors tend to be mostly homogeneous and hypointense relative to the liver. A minority of tumors may be heterogeneous and isointense. On T2-weighted images, the tumors are commonly heterogeneous and, most often, hyperintense with respect to the liver. [21, 17] The images below demonstrate the hypointensity and hyperintensity of T1- and T2-weighted images of fibrolamellar carcinoma. [12, 10, 19]\nT1-weighted magnetic resonance image of the liver in a patient with fibrolamellar carcinoma shows a lobulated and well-defined mass with a relatively homogeneous, hypointense appearance.\nT2-weighted magnetic resonance image (same patient as in the previous image) shows a fibrolamellar carcinoma with increased signal intensity relative to that of the surrounding liver.\nThe central scar usually appears hypointense on all images obtained with all sequences. The appearance of the scar on MR images can be useful in differentiating fibrolamellar carcinomas from FNH, because scars in FNH tend to be hyperintense on T2-weighted images. Both fibrolamellar carcinoma and FNH are characterized primarily by generally subtle deviations of signal intensities on precontrast T1-weighted images and T2-weighted images, as compared to background liver parenchyma, arterial hyperenhancement, and the presence of a central scar. Imaging features that may favor fibrolamellar carcinoma over FNH on MRI include the greater heterogeneity of lesion texture of fibrolamellar carcinoma, the hypointensity of the central scar of fibrolamellar carcinoma on T2-weighted images, and portal venous hypoenhancement. [10, 18]\nFibrolamellar carcinomas typically do not contain intracellular fat; therefore, the presence of fat on fat-saturated, in-phase, and out-of-phase MRI scans suggests HCC or an adenoma. [19]\nOn gadolinium-enhanced magnetic resonance images, the enhancement patterns seen in fibrolamellar carcinomas are similar to those seen on contrast-enhanced CT scans. Early heterogeneous enhancement occurs during the arterial phase and progresses to more homogeneous enhancement during delayed phases. The central scar does not enhance during the arterial phase, but it may demonstrate mild enhancement in the later portal or equilibrium phases.\nGadolinium-based contrast agents have been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). NSF/NFD is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness.\nMRI is sensitive in the detection of intrahepatic masses of fibrolamellar carcinoma, and the modality may be slightly more sensitive than CT scanning in depicting multiple recurrent intrahepatic lesions. Although metastatic lymphadenopathy can be seen on MRI scans, CT scanning is probably more useful for detecting distant metastases, particularly in the lungs. CT scanning is also relatively less expensive than MRI; therefore, CT scanning is most appropriate for initial imaging and follow-up in most patients.\nMRI is most useful in the characterization of indeterminate masses seen on CT scans. Often, MRI features of tumors can help confirm a suggested diagnosis of fibrolamellar carcinoma, or they can exclude the possibility of other entities such, as FNH or HCC.\nAs with CT scanning, MRI is not entirely specific for fibrolamellar carcinoma, and other liver lesions (eg, adenoma, HCC, FNH) can occasionally mimic the appearance of fibrolamellar carcinoma. For instance, the hyperintense scar that is usually associated with FNH has been reported in at least one patient with fibrolamellar carcinoma.\nAbdominal ultrasonograms are often obtained for the initial evaluation of patients with abdominal pain; therefore, ultrasonography may be the first imaging study available in patients with fibrolamellar carcinoma. On ultrasonograms, the primary tumor can be seen as a solitary, well-defined hepatic mass with a heterogeneous echotexture (as demonstrated in the image below). The tumor's scar may be seen as a central, hyperechoic structure, and calcification may be represented by an echogenic focus with shadowing. [8]\nUltrasonogram shows a heterogeneous echotexture mass in the liver that corresponds to a fibrolamellar carcinoma.\nBecause the primary intrahepatic tumors of fibrolamellar carcinoma are often large, the likelihood of detecting the mass with ultrasonography is high. However, ultrasonography is less sensitive than CT scanning in depicting tumoral characteristics, such as scars and calcifications, which aid in the differentiation of fibrolamellar carcinoma from other intrahepatic tumors. Ultrasonography is less accurate than CT scanning in staging extrahepatic disease. After the initial detection of the intrahepatic mass with ultrasonography, further evaluation with CT scanning or MRI is usually necessary for the definitive diagnosis and staging of fibrolamellar carcinoma.\nAlthough, in a young patient, a large intrahepatic mass with a central scar is suggestive of fibrolamellar carcinoma, other entities (eg, FNH, hepatocellular adenoma, possibly giant cavernous hemangioma) may have a similar appearance on ultrasonograms and result in false-positive findings. False-negative ultrasonographic findings may occur in patients with obesity, underlying cirrhosis or fatty infiltration (which is not typical in patients with fibrolamellar carcinoma), or a body habitus that may obscure good visualization of the liver during ultrasonography.\nRadionuclide imaging studies are not useful in the detection of fibrolamellar carcinoma. However, in some patients, these studies may be useful in the differential diagnosis of the tumors.\nOn sulfur-colloid liver-spleen scans, fibrolamellar carcinomas appear as photopenic defects in the liver; the masses demonstrate decreased activity. On scans obtained with red blood cells labeled with technetium-99m (99mTc), fibrolamellar carcinomas typically demonstrate increased activity on early arterial-phase images. This finding is followed by decreased activity with respect to the surrounding liver on delayed-phase images.\nIn difficult or equivocal cases, radionuclide sulfur-colloid images or images obtained with labeled red blood cells may provide information that increases the level of confidence in the diagnosis of fibrolamellar carcinoma. However, these studies are not useful in detecting or staging fibrolamellar carcinoma.\nSulfur-colloid liver-spleen scans can demonstrate photopenic lesions in the liver with many entities, including benign and malignant liver tumors, cysts, and abscesses; therefore, false-positive results are possible. False-negative sulfur-colloid scans may occur when diffuse hepatocellular dysfunction, such as cirrhosis, results in poor tracer uptake in a noncancerous liver.\nOther liver tumors may have uptake patterns on labeled red blood cell scans that are similar to the uptake pattern of fibrolamellar carcinoma; therefore, false-positive findings can result. The tumors may demonstrate normal activity levels, resulting in a false-negative finding.\nAngiography is usually not beneficial in the detection, staging, or evaluation of fibrolamellar carcinoma. If obtained, angiograms demonstrate the primary tumor as a hypervascular mass (as seen in the image below) with multiple feeding arteries, a dense tumor blush, and an avascular central scar.\nWhat is fibrolamellar hepatocellular carcinoma?\nWhat is the role of CT scans in the workup of fibrolamellar hepatocellular carcinoma?\nWhat is the role of MRI in the workup of fibrolamellar hepatocellular carcinoma?\nWhat is the role of ultrasonography in the workup of fibrolamellar hepatocellular carcinoma?\nWhat is the role of nuclear imaging in the workup of fibrolamellar hepatocellular carcinoma?\nWhich findings on radiography are characteristic of fibrolamellar hepatocellular carcinoma?\nWhich findings on CT scans are characteristic of fibrolamellar hepatocellular carcinoma?\nWhat is the accuracy of CT scans for the evaluation of fibrolamellar hepatocellular carcinoma?\nWhich findings on MRI are characteristic of fibrolamellar hepatocellular carcinoma?\nWhat is the accuracy of MRI for the evaluation of fibrolamellar hepatocellular carcinoma?\nWhich findings on ultrasonography are characteristic of fibrolamellar hepatocellular carcinoma?\nWhich findings on radionuclide imaging are characteristic of fibrolamellar hepatocellular carcinoma?\nWhich findings on angiography are characteristic of fibrolamellar hepatocellular carcinoma?\nLiu S, Chan KW, Wang B, Qiao L. Fibrolamellar hepatocellular carcinoma. Am J Gastroenterol. 2009 Oct. 104(10):2617-24; quiz 2625. [Medline].\nChakrabarti S, Tella SH, Kommalapati A, Huffman BM, Yadav S, Riaz IB, et al. Clinicopathological features and outcomes of fibrolamellar hepatocellular carcinoma. J Gastrointest Oncol. 2019 Jun. 10 (3):554-561. [Medline].\nAng CS, Kelley RK, Choti MA, Cosgrove DP, Chou JF, Klimstra D, et al. Clinicopathologic characteristics and survival outcomes of patients with fibrolamellar carcinoma: data from the fibrolamellar carcinoma consortium. Gastrointest Cancer Res. 2013 Jan. 6 (1):3-9. [Medline].\nEl-Serag HB. Epidemiology of hepatocellular carcinoma in USA. Hepatol Res. 2007 Sep. 37 Suppl 2:S88-94. [Medline].\nEggert T, McGlynn KA, Duffy A, Manns MP, Greten TF, Altekruse SF. Epidemiology of fibrolamellar hepatocellular carcinoma in the USA, 2000-10. Gut. 2013 Nov. 62 (11):1667-8. [Medline].\nWood WJ, Rawlings M, Evans H, et al. Hepatocellular carcinoma: importance of histologic classification as a prognostic factor. Am J Surg. 1988 May. 155(5):663-6. [Medline].\nMalouf G, Falissard B, Azoulay D, et al. Is histological diagnosis of primary liver carcinomas with fibrous stroma reproducible among experts?. J Clin Pathol. 2009 Jun. 62(6):519-24. [Medline].\nLafaro KJ, Pawlik TM. Fibrolamellar hepatocellular carcinoma: current clinical perspectives. J Hepatocell Carcinoma. 2015. 2:151-7. [Medline].\nTitelbaum DS, Burke DR, Meranze SG, et al. Fibrolamellar hepatocellular carcinoma: pitfalls in nonoperative diagnosis. Radiology. 1988 Apr. 167(1):25-30. [Medline]. [Full Text].\nGaneshan D, Szklaruk J, Kundra V, Kaseb A, Rashid A, Elsayes KM. Imaging features of fibrolamellar hepatocellular carcinoma. AJR Am J Roentgenol. 2014 Mar. 202 (3):544-52. [Medline].\nChun YS, Zimmitti G. Fibrolamellar variant of hepatocellular carcinoma. Recent Results Cancer Res. 2013. 190:101-10. [Medline].\nDo RK, McErlean A, Ang CS, DeMatteo RP, Abou-Alfa GK. CT and MRI of primary and metastatic fibrolamellar carcinoma: a case series of 37 patients. Br J Radiol. 2014 Aug. 87 (1040):20140024. [Medline].\nValentino PL, Ling SC, Ng VL, John P, Bonasoni P, Castro DA, et al. The role of diagnostic imaging and liver biopsy in the diagnosis of focal nodular hyperplasia in children. Liver Int. 2013 Jun 12. [Medline].\nBlachar A, Federle MP, Ferris JV, Lacomis JM, Waltz JS, Armfield DR, et al. Radiologists' performance in the diagnosis of liver tumors with central scars by using specific CT criteria. Radiology. 2002 May. 223 (2):532-9. [Medline].\nEggert T, McGlynn KA, Duffy A, Manns MP, Greten TF, Altekruse SF. Fibrolamellar hepatocellular carcinoma in the USA, 2000-2010: A detailed report on frequency, treatment and outcome based on the Surveillance, Epidemiology, and End Results database. United European Gastroenterol J. 2013 Oct. 1 (5):351-7. [Medline].\nIchikawa T, Federle MP, Grazioli L, Madariaga J, Nalesnik M, Marsh W. Fibrolamellar hepatocellular carcinoma: imaging and pathologic findings in 31 recent cases. Radiology. 1999 Nov. 213 (2):352-61. [Medline].\nBartolozzi C, Battaglia V, Bozzi E. HCC diagnosis with liver-specific MRI--close to histopathology. Dig Dis. 2009. 27(2):125-30. [Medline].\nPalm V, Sheng R, Mayer P, Weiss KH, Springfeld C, Mehrabi A, et al. Imaging features of fibrolamellar hepatocellular carcinoma in gadoxetic acid-enhanced MRI. Cancer Imaging. 2018 Mar 1. 18 (1):9. [Medline]. [Full Text].\nAlexander LF, Harri P, Little B, Moreno CC, Mittal PK. Magnetic Resonance Imaging of Primary Hepatic Malignancies in Patients With and Without Chronic Liver Disease: A Pictorial Review. Cureus. 2017 Aug 4. 9 (8):e1539. [Medline]. [Full Text].\nDesai A, Sandhu S, Lai JP, Sandhu DS. Hepatocellular carcinoma in non-cirrhotic liver: A comprehensive review. World J Hepatol. 2019 Jan 27. 11 (1):1-18. [Medline]. [Full Text].\nMcLarney JK, Rucker PT, Bender GN, et al. Fibrolamellar carcinoma of the liver: radiologic-pathologic correlation. Radiographics. 1999 Mar-Apr. 19(2):453-71. [Medline]. [Full Text].\nLiu S, Wah Chan K, Tong J, Wang Y, Wang B, Qiao L. PET-CT scan is a valuable modality in the diagnosis of fibrolamellar hepatocellular carcinoma: a case report and a summary of recent literature. QJM. 2011 Jun. 104 (6):477-83. [Medline].\nPhotograph of a resected gross pathologic specimen of fibrolamellar carcinoma shows a lobulated, tan-colored mass with a stellate, central scar.\nW Ross Stevens, MD\nW Ross Stevens, MD is a member of the following medical societies: American College of Radiology, American Medical Association, American Roentgen Ray Society, Illinois State Medical Society, Radiological Society of North America\nBernard D Coombs, MB, ChB, PhD Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand\nUdo P Schmiedl, MD, PhD Clinical Professor, Department of Radiology, University of Washington; Consulting Staff, Swedish Medical Center, University of Washington Medical Center, Seattle Radiologists\nUdo P Schmiedl, MD, PhD is a member of the following medical societies: American College of Radiology, Radiological Society of North America\nJohn Karani, MBBS, FRCR Clinical Director of Radiology and Consultant Radiologist, Department of Radiology, King's College Hospital, UK\nJohn Karani, MBBS, FRCR is a member of the following medical societies: British Institute of Radiology, Radiological Society of North America, Royal College of Radiologists, Cardiovascular and Interventional Radiological Society of Europe, European Society of Radiology, European Society of Gastrointestinal and Abdominal Radiology, British Society of Interventional Radiology\nJohn L Haddad, MD Clinical Associate Professor, Department of Radiology, Weill Medical College of Cornell University; Director of Body MRI, Department of Radiology, Methodist Hospital in Houston\nJohn L Haddad, MD is a member of the following medical societies: American College of Radiology, American Medical Association, and Radiological Society of North America\nencoded search term (Fibrolamellar Hepatocellular Carcinoma Imaging) and Fibrolamellar Hepatocellular Carcinoma Imaging\nHepatocellular Carcinoma (HCC)\nPrimary Hepatic Carcinoma\nFibrolamellar Carcinoma\nHepatocellular Carcinoma Imaging\nHepatic Chemoembolization (TACE) Imaging\nHepatocellular Carcinoma: New Treatment Options and Sequencing Considerations\nHepatocellular Carcinoma Staging\nFrom Survivorship to End-of-Life Care\nInfections Account for 13% of All Cancer Cases\nLower Hepatoma Risk With Earlier ALT Normalization During HBV Treatment\nTop News From ESMO 2019: Slideshow\nNews Lower Hepatoma Risk With Earlier ALT Normalization During HBV Treatment\nNews Improved Survival Seen in Hepatoma Patients After HCV Cure\nNews No Survival Difference Between Re-Resection, Percutaneous RFA in Recurrent HCC\nProcedures Hepatocellular Carcinoma (HCC)"