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