los angeles society of pathologistslasop.com/pgs/hdouts/2006-03_case4.pdf · clinical features •...
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Los Angeles Society of Pathologists
Najeeb S. Alshak, M.D.Kaiser Permanente, Los Angeles
March 2006
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Infantile Hemangioendothelioma
CASE #4
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Clinical Features
• 18% of all liver tumor in children <21 y• 40% of all benign liver tumor < 21 y• 40% of all liver tumor in first 2 y of life• 70% of all benign liver tumor in first 2 y• 90% occur in first 6 month• 33% occur in first month • Female > male 2/1• White = black
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Clinical Features• 40% are asymptomatic• Abdominal mass due to hepatomegaly• Congestive high output, arteriovenous shunt,
heart failure• Consumptive coagulopathy/DIC (Kasabach-
Merritt syndrome)• Anemia• Hyperbilirubinemia• Hemangiomata in other organs (skin, GI tract,
trachea, pleura, heart, adrenal, pleura)• Rarely rupture (hemoperitoneum)
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Gross Findings
• Single tumor in 55%, 0.5 cm to 13 cm
• Multifocal, 2-30 nodules
• Soft to firm, tan-white to dark brown
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Microscopic Findings• Vascular channels lined by single thin
layer• Bland flat to plump endothelial cells in
supporting fibrous stroma• Small bile ducts• Well demarcated margin in 65% vs.
infiltrating adjacent parenchyma (entrapped hepatocytes)
• Pseudoglandular, degeneration, transformation to bile ducts
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Microscopic Findings
• Cavernous change in 60% (central and periphery)
• Extramedullary hematopoiesis within the vascular channels and stroma
• Involutional change: fibrosis, calcifications and myxoid change
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Immunohistochemistry
• Factor VIII• CD31• CD34• Vimentin• Ulex Europaeus• Lecitin
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Molecular Findings
• 75% diploid
• 15% aneuploid, skin lesions, bad prognosis
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Differential Diagnosis
• Cavernous hemangioma• Mesenchymal hamartoma• Angiosarcoma• Epithelioid hemangioendothelioma• Embryonal (undifferentiated) sarcoma• Hepatoblastoma
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Hepatoblastoma
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Hepatoblastoma
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Hepatoblastoma
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Cavernous Hemangioma
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Cavernous Hemangioma
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Epithelioid Hemangioendothelioma
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Angiosarcoma
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Mesenchymal Hamartoma
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Mesenchymal Hamartoma
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Undifferentiated (Embryonal) Sarcoma
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Undifferentiated (Embryonal) Sarcoma
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Treatment
• Conservative/ corticosteroids and Vincristine, Digitalis, Interferon
• Surgery• Hepatic artery ligation• Transarterial ambolization• Liver transplant
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Prognosis
• Spontaneous regression in up to 10%
• 70% overall survival
• Death 1 month after diagnosis
• Transformation to angiosarcoma