4.
Please wait for the figures to download
3.4.
5.
Diagnosis: Angiosarcoma (Also see Case of the Month February/March 2001)
Histopathology: A scalp skin biopsy showed a deeply infiltrative tumor composed of basophilic cells with some areas containing slightly angulated and richly anastomotic apparent vascular spaces (Figure 3). The lesion was solid in some areas. There were CD31 positive cells in the biopsy, even extending into the subcutis in one area (Figures 4,5).
Discussion: Angiosarcomas are uncommon malignant vascular tumors that comprise less than 2% of all soft tissue sarcomas. Although they can arise in any organ of the body, they are most commonly found in the skin and superficial soft tissue (60%), but also in the breast and liver (1,2). Fifty percent of all cutaneous angiosarcomas occur in the head and neck region (3). It affects men more frequently than women and tends to be an aggressive malignancy with extensive rapid growth and often has local recurrence despite multifaceted therapy.
Cutaneous Angiosarcoma initially presents as nonspecific erythematous and violaceous plaques or nodules on the face and scalp that can mimic rosacea, cellulitis or erysipelas. In part, the poor outcome associated with cutaneous angiosarcoma is secondary to the frequent delay in establishing a diagnosis due to its sometimes subtle and varied clinical features. The tumor is often advanced by the time the nature of the lesion is established. Larger tumors are often complicated by extensive ulceration (4). Facial angiosarcoma most commonly metastasizes to the regional lymph nodes, lungs and liver.
Histologically these tumors tend to be highly infiltrative tumors with marked cytologic atypia and mitotic activity. They are composed atypical spindle-shaped or epithelioid cells forming slit-like vascular channels as the cells attempt to organize to form blood vessels. Angiosarcoma may arise from the vascular or lymphatic system, or both (5). Immunohistochemically, tumor cells stain positively for CD31 and vimentin markers.
Retrospective studies suggest that patients treated with surgical debridement followed with radiotherapy have the best chance for long term tumor free survival (1). However, surgical margins are difficult to define and local recurrence is common. It appears that age, sex, location and clinical appearance have no statistical correlation with survival, even histologic differentiation of the initial tumor appears to have little prognostic value (4). The only significant indicator of prognosis is tumor size at initial presentation (1,4) emphasizing the need for close follow up of bruise-like lesions on the head and neck of elderly patients. Despite therapy, the prognosis is grim with less than half patients surviving one year after diagnosis. The five-year survival rate is only 12-24% (1,4)
RFERENCES:
1. Schoen FJ. Blood Vessels in Pathologic Basis of Disease ed Cotran, Kumar, Robbins. 5th edition. 1994. p510 511.
2. Mark RJ, Poen JC, Tran LM. Angiosarcoma. A report of 67 patients and a review of the literature. Cancer 1996. 77:2400-6.
3. Morales PH, Lindberg RD, Barkley HT. Soft tissue angiosarcomas. Int J Radiat Oncol Biol Phy 1981. 7:1655 9.
4. Holden CA, Spittle MF, Jones EW. Angiosarcoma of the face and scalp, prognosis and treatment. Cancer 1987. 59:1046-57.
5. Orchard GE, Zelger B, Jones EW, Jones RR. An immunohistochemical assessment of 19 cases of cutaneous angiosarcoma. Histopathology 1996. 28:235-49.
My thanks to Drs. Saadia Raza and David Sheinbein for their assistance
in the preparation of this case.
