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Liver and intrahepatic bile ducts - tumor

Other malignancies

Infantile hemangioendothelioma / hemangioma


Reviewers: Deepali Jain, M.D. (see Reviewers page)
Revised: 9 January 2013, last major update February 2012
Copyright: (c) 2004-2013, PathologyOutlines.com, Inc.

General
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● Most common hepatic mesenchymal tumor in childhood (20% of all pediatric hepatic tumors)

Clinical features
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● 90% are <6 months old at diagnosis, slight female predominance
● 10-40% have coexisting cutaneous cavernous hemangiomas
● 50% are incidental findings at autopsy
● Symptoms: hepatic mass (48%), high output cardiac failure due to shunting through tumor (15%); also Kasabach-Merritt syndrome (bleeding diathesis due to platelet sequestration and severe thrombocytopenia); may be asymptomatic
● Associations: chromosomal abnormalities, developmental abnormalities, hemangiomas, mesenchymal hamartoma, Wilm's tumor (Virchows Arch A Pathol Anat Histopathol 1988;413:463)

Laboratory and Radiology
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● Normal AFP (usually), rarely elevated due to secretion from entrapped or nearby hepatocytes (Hum Pathol 2010;41:763)
● Xray: multiple small nodules

Treatment and prognosis
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● Spontaneous involution in 5-10% at 6-8 months; resection if solitary, otherwise steroids, interferon, radiation therapy, embolization or transplantation (World J Surg 2009;33:597)
● Poor prognostic factors: congestive heart failure, jaundice, multiple nodules, lack of cavernous differentiation
● 70% survival (almost always have benign behavior)
● Deaths (when they occur) are usually within 1 month of diagnosis, and due to congestive heart failure or platelet consumption leading to bleeding diathesis or massive hemoperitoneum

Case reports
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● 56 year old woman with breast DCIS and liver mass (Arch Pathol Lab Med 2001;125:931)
● With pulmonary metastases (J Pediatr Surg 2006;41:e49)

Gross description
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● Solitary or multiple, mean 4 cm (range 0.1 to 15 cm) and circumscribed but not encapsulated
● White-red-tan, soft and spongy
● Larger nodules may have hemorrhagic, fibrotic or calcified central areas

Gross images
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Various images

Micro description
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● Well-demarcated or infiltrative (35%)
Pure type 1 change: 80%, orderly proliferation of small, capillary-like vascular spaces, relatively bloodless, may be dilated (particularly centrally) and slightly irregular; lined by bland or plump endothelial cells that may occlude the lumen; vascular channels separated by variable connective tissue; may have interspersed small bile ducts; extramedullary hematopoiesis in 60%, often in vascular lumina; often trapped hepatocytes at periphery; large lesions show thrombosis, fibrosis, myxoid change and calcification; no / rare mitotic figures and no malignant spindle cell component
Type 2 change: equivalent to angiosarcoma (with aggressive behavior) with irregular branching vascular structures lined by pleomorphic, hyperchromatic endothelial cells, frequent mitotic activity

Micro images
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Various images


Bland cells with small / medium nuclei and nucleoli, factor VIII related antigen

Positive stains
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● Factor VIII related antigen, CD31, CD34 and GLUT1 (Hum Pathol 2004;35:200)

Differential diagnosis
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Angiosarcoma: adequate sampling is important; has solid sarcomatous areas, vascular and sinusoidal permeation, marked pleomorphism; may have history of exposure to toxins
Cavernous hemangioma: less common in children, lacks peripheral small vascular proliferation
Epithelioid hemangioendothelioma: intracellular vascular lumina containing RBCs
Hepatic vascular malformation with capillary proliferation: typically single large mass, malformed, irregular large vessels and Glut1-
Mesenchymal hamartoma: primitive mesenchymal stroma, not a vascular tumor

End of Liver and intrahepatic bile ducts - tumor > Other malignancies > Infantile hemangioendothelioma / hemangioma


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