Composite haemangioendothelioma

Definition

A rare vascular tumour composed of histologically benign, intermediate and malignant elements.

Clinical features

Most cases present on the extremities of young adults as cutaneous or subcutaneous lesions. Other sites include the oral cavity. There is an association with Mafucci syndrome.

Histopathology

The dominant component closely resembles retiform or epithelioid haemangioendothelioma. The retiform component consists of slender vascular channels are lined by hyperchromatic hobnail endothelial cells. An epithelioid component consists of cords or nests of epithelioid cells within a myxohyaline stroma. Other components include spindle cell haemangioma, lymphangioma, cavernous haemangioma, AV malformation or diffuse haemangioma. Any angiosarcomatous component is small.

Immunohistochemistry

	At least two of CD31, CD34 and Factor VIIIRA	4/41
	D2-40	0/41

Management

Wide local excision.

Prognosis

The tumour recurs in half of all cases and may be locally aggressive. Metastases are rare. An angiosarcoma-like component does not seem to worsen the prognosis. There have been no reported fatalities.

References

1 Fukunaga M, Suzuki K, Saegusa N, et al. Composite hemangioendothelioma: report of 5 cases including one with associated Maffucci syndrome. Am J Surg Pathol 2007; 31:1567-72

This page last revised 15.2.2008