Liver & intrahepatic bile ducts

Other malignancies

Infantile hemangioendothelioma / hemangioma



Topic Completed: 1 February 2012

Minor changes: 11 June 2021

Copyright: 2004-2021, PathologyOutlines.com, Inc.

PubMed search: Infantile hemangioendothelioma[TI] OR infantile hemangioma[TI] liver


Deepali Jain, M.D.
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Cite this page: Jain D. Infantile hemangioendothelioma / hemangioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/livertumorIHE.html. Accessed December 3rd, 2021.
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, Wilms 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
Case reports
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)
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

Case #432

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Differential diagnosis
  • 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
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