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

Authors: Deepali Jain, M.D. (see Authors page)

Revised: 2 August 2017, last major update February 2012

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PubMed search: Infantile hemangioendothelioma [title]

Cite this page: Liver and intrahepatic bile ducts - tumor - Infantile hemangioendothelioma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/livertumorIHE.html. Accessed November 24th, 2017.
Definition / general
  • Most common hepatic mesenchymal tumor in childhood (20% of all pediatric hepatic tumors)
Clinical features
  • 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
  • Normal AFP (usually), rarely elevated due to secretion from entrapped or nearby hepatocytes (Hum Pathol 2010;41:763)
Radiology description
  • Xray: multiple small nodules
Prognostic factors
  • 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
Treatment
  • 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)
Case reports
Gross description
  • 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
Microscopic (histologic) description
  • 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
Microscopic (histologic) images

Images hosted on PathOut server, Case of the Week #432:

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Differential diagnosis