Cutaneous angiosarcoma

Aetiology

Cutaneous angiosarcomas arise in three settings:

Clinical features

Post-radiation angiosarcomas often cover large ares of skin and are multifocal.

Histopathology

The tumour infiltrates dermis and subcutis. There is a wide spectrum of differentiation. Well differentiated tumours form vessels that grow ins diffusely infiltrative manner between collagen fibres. The endothelial cells are multilayered and show cytological atypia. Poorly differentiated tumours consist of solid sheets of cells which may be spindled or epithelioid. They show severe cytological atypia, a high mitotic rate and necrosis. Vasoformation by be evidenced by intracytoplasmic lumina which may contain erythrocytes. In proximity to the angiosarcoma, there may be atypical vascular lesions.

Immunohistochemistry

 

CD31

positive

 

CD34

positive

Factor VIIIRA

positive

Cytokeratin

may be positive

D2-40

may be positive

CD30

1/12, 1/13

   

Differential diagnosis

Prognosis

Sporadic and lymphoedema-associated angiosarcoma has a high metastatic rate and a five-year survival or only about 10%. The behaviour of post-radiation angiosarcomas is less certain, with a high rate of local recurrence but possibly less frequent metastases.

References

1 Brenn T,Fletcher CD Postradiation vascular proliferations: an increasing problem. Histopathology 2006; 48:106-14

2 Weed BR,Folpe AL Cutaneous CD30-positive epithelioid angiosarcoma following breast-conserving therapy and irradiation: a potential diagnostic pitfall. Am J Dermatopathol 2008; 30:370-2

3 Unpublished case seen by myself and the pathology department, Christie Hospital, Manchester

This page last revised 22.12.2008.

©SMUHT/PW Bishop