Probably not distinct entity, but rather descriptive designation with associated clinical correlates Etiology/Pathogenesis • Majority of cases have no known cause or disease association • Some associated with hypersensitivity reactions, parasitic infection,…
Often submitted for frozen section to rule out metastasis Microscopic • Uniformly sized tubules and acini with rounded outlines • Single layer of cuboidal to columnar cells that lack atypia, hyperchromasia, mitoses…
Results in hepatosplenomegaly and pancytopenia • Variable disease progression • Enzyme replacement therapy represents mainstay therapy Early diagnosis is crucial to improving outcome Microscopic • Accumulation of glucocerebroside in Kupffer cells and portal…
Autosomal recessive Classification • Types A and B characterized by acid sphingomyelinase deficiency • Types C and D harbor cholesterol metabolism defect Etiology/Pathogenesis • Inherited lysosomal hydrolase deficiency leads to accumulation of sphingolipid…
Typically 1-3 mm • No fibrous septa between nodules But focal sinusoidal or periportal fibrosis can be seen • Inflammation is absent Bile ducts and hepatic arterioles are normal Top Differential Diagnoses…
Once known etiologies are excluded, then considered idiopathic • By mid-2000s, idiopathic neonatal hepatitis (INH) comprised only 15-30% of neonatal cholestasis due to increased ability to detect and diagnose metabolic and…
Often classified as having high-grade dysplasia given architectural complexity • Neoplastic cells have abundant granular eosinophilic cytoplasm with large nuclei, prominent nucleoli • Both intracytoplasmic and intercellular lumina found, many containing mucin,…
Pale, enlarged hepatocytes may be mistaken for normal or interpreted as fixation artifact • Fatty liver disease Pale, enlarged hepatocytes may be misinterpreted as ballooning hepatocyte degeneration • Glycogen storage disease No…
Characterized by ground-glass or smudged nuclei with margination of chromatin • Multinucleated cells with nuclear molding are less common compared with mucocutaneous HSV infections • Immunohistochemistry with antibodies directed against HSV1/HSV2 confirm…
Infected hepatocytes may be slightly enlarged and multinucleated May show hemorrhagic appearance in areas of necrosis • Varicella-zoster virus hepatitis • CMV hepatitis Gross AppearanceThis liver specimen from an autopsy case shows…
