hemangioendothelioma

Epidemiology

• rare malignant tumor
• sffects fewer than 300 patients per year in the United States
• accounts for ~1% of all vascular neoplasms

Pathology

• bone involvement
• tumors express a wide variety of ligands & receptors for VEGF
• ~15% of patients with epithelioid hemangioendothelioma die of the disease
• tissue biopsy

Microscopic pathology

• immunohistochemical staining
  • tumor cells positive for vascular endothelial markers CD31 & FLI1
  • staining for CD117 may show scattered mast cells

Genetics

• chromosomal translocation involving chromosome 1 & chromosome 3

Radiology

• computed tomography or magnetic resonance imaging

Management

• treted as low-to-intermediate-grade angiosarcoma
• bevacizumab of benefit in a minority of patients
• monotherapy with sunitinib, thalidomide, lenalidomide, or IFN-alpha with reported benefits
• gemcitabine, carboplatin-etoposide, & liposomal doxorubicin of reported benefit
• pallitative radiation therapy for bone pain

More general terms

• vascular tissue neoplasm; angioma

Patient information

hemangioendothelioma patient information