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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
Interactions
disease interactions
General
vascular tissue neoplasm; angioma
References
- Choy E, Attar EC, Oh KS, Huang AJ, Kerr DA.
Case 33-2014 - A 60-Year-Old Man with Bone Pain.
N Engl J Med 2014; 371:1630-1640. October 23, 2014
PMID: 25337753
http://www.nejm.org/doi/full/10.1056/NEJMcpc1310000