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composite hemangioendothelioma

Monday 1 October 2007

Composite hemangioendothelioma (HE) is a low-grade malignant vascular tumor showing varying combinations of benign, low-grade malignant, and malignant vascular components.

The predominant histologic components are histologically identical to epithelioid HE and retiform HE.

Composite HE is regarded as a low-grade malignant vascular tumor (HE), with significant potential for local recurrence, but little if any potential for distant metastasis.

Composite haemangioendothelioma is an exceedingly uncommon vascular tumour characterized, in most cases, by a complex admixture of histologically benign, intermediate and malignant components, most often consisting of retiform haemangioendothelioma and epithelioid haemangioendothelioma.

However, a significant proportion of cases also contain angiosarcoma-like areas. These tumours, which are commonest in the distal extremities, often recur locally but only rarely metastasize.

Synopsis

- 4 females for 1 male
- median age of 43.4 years (range, 22 to 75 y)
- dermis and/or subcutis
- +/- congenital tumors
- size : 1.5 to 30 cm
- predominant components: retiform HE and epithelioid HE
- angiosarcomalike areas
- lymphangiomalike areas
- +/- areas of spindle cell hemangioma, cavernous hemangioma, or arteriovenous malformation
- +/- Kasabach-Merritt syndrome

Immunohistochemically

- CD31
- CD34
- factor VIII-related antigen

Predisposition

- Maffucci syndrom

References

- Fukunaga M, Suzuki K, Saegusa N, Folpe AL. Composite Hemangioendothelioma: Report of 5 Cases Including One With Associated Maffucci Syndrome. Am J Surg Pathol. 2007 Oct;31(10):1567-1572. PMID: #17895759#