Therapeutics in Dermatology
A reference textbook in dermatology
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Cutaneous angiosarcoma

24 July 2012, by RENAUD-VILMER C.

Angiosarcomas (AS) are a rare kind of tumour with an aggressive course and a poor prognosis that account for approximately 1 - 2 % of soft tissue sarcomas [22, 24]. They usually develop in the skin and superficial tissue (60 % of cases) but they can also develop in the internal organs [24]. Growth of cutaneous AS may be encouraged by the presence of a congenital or acquired chronic lymphoedema (the most common type of AS is Stewart-Treves syndrome, which develops in chronic lymphoedema of the arm after mastectomy, axiliary dissection and radiotherapy to treat breast cancer), irradiation sites, chronic ulceration (arteriovenous fistula in kidney transplant patients) etc. The assertion that the current conservative breast cancer treatments with radiotherapy play a role in encouraging angiosarcomas to develop is controversial [1, 2, 4, 10, 17] (Figure 1): indeed, one study [17] showed an identical prevalence (5 in 10,000) to that of AS developing when the breast had not had this treatment, while other studies, on the contrary, report that radiotherapy during these conservative treatment approaches does play a role in encouraging angiosarcoma (OR: 11:6). In the latter case, the latency period is shorter. However, approximately 78 - 95 % of cutaneous AS arise with no predisposing factors. The role of the HHV-8 virus has not been confirmed. Cutaneous AS usually affect the head and the neck (with scalp involvement in 50 % of cases), and occur most often in patients over the age of 60, with a M/F sex ratio of 2.5 – 3 to 1 [11] (Figure 2 and Figure 3). It has been suggested that UV rays may be involved in head and neck localisations. The prognosis of cutaneous AS remains very severe, with five-year survival rates of 10 - 35 % [18, 19, 21-23]. Furthermore, AS is a multifocal tumour which spreads beyond the clinically observable edges of the lesions with histological occult extension, total surgical excision is often not achieved. In addition, excision attempts may be impeded by the presence of vital structures when the tumour is located in the head or the neck. There is often local recurrence, mainly in the first two years after the initial treatment, and this seems to correlate to tumour size. However, for AS of the extremities it is interesting to note that amputation does not alter the overall survival rate [12, 22] and that wide radical excisions do not avoid recurrence, which arises in 20 - 60 % of cases [22]. In multivariate analyses [21, 24], the factors used to evaluate prognosis are tumour size and thickness, mitotic index, and the surgical margins.

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