Post on 23-Aug-2020
Los Angeles Society of Pathologists
Najeeb S. Alshak, M.D.Kaiser Permanente, Los Angeles
March 2006
Infantile Hemangioendothelioma
CASE #4
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
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)
Gross Findings
• Single tumor in 55%, 0.5 cm to 13 cm
• Multifocal, 2-30 nodules
• Soft to firm, tan-white to dark brown
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
Microscopic Findings
• Cavernous change in 60% (central and periphery)
• Extramedullary hematopoiesis within the vascular channels and stroma
• Involutional change: fibrosis, calcifications and myxoid change
Immunohistochemistry
• Factor VIII• CD31• CD34• Vimentin• Ulex Europaeus• Lecitin
Molecular Findings
• 75% diploid
• 15% aneuploid, skin lesions, bad prognosis
Differential Diagnosis
• Cavernous hemangioma• Mesenchymal hamartoma• Angiosarcoma• Epithelioid hemangioendothelioma• Embryonal (undifferentiated) sarcoma• Hepatoblastoma
Hepatoblastoma
Hepatoblastoma
Hepatoblastoma
Cavernous Hemangioma
Cavernous Hemangioma
Epithelioid Hemangioendothelioma
Angiosarcoma
Mesenchymal Hamartoma
Mesenchymal Hamartoma
Undifferentiated (Embryonal) Sarcoma
Undifferentiated (Embryonal) Sarcoma
Treatment
• Conservative/ corticosteroids and Vincristine, Digitalis, Interferon
• Surgery• Hepatic artery ligation• Transarterial ambolization• Liver transplant
Prognosis
• Spontaneous regression in up to 10%
• 70% overall survival
• Death 1 month after diagnosis
• Transformation to angiosarcoma