Angiosarcoma of the face and scalp

ARTICLE

Histopathological findings

Histopathological examination showed invasion of the dermis by pleomorphic cells that line irregular channels which dissect the connective tissue. Some channels contain red blood cells. The pleomorphic cells are large with hyperchromatic nuclei and protrude into the vascular lumina. Immunohistochemical study demonstrated positivity of the neoplastic cells for factor VIII related antigen, CD31 and CD34. Moderately differentiated angiosarcoma was diagnosed.

Clinical course and treatment

Chest X-rays, computerized tomography of the chest and head and neck, ultrasonography of the abdomen and total body scintigraphy showed no evidence of regional or systemic metastases.

We were confronted with an extensive angiosarcoma of the face and scalp without evidence of systemic involvement. Surgical resection was impossible. Electronbeam radiation therapy was used at a total dose of 60 Gy over a duration of 6 weeks. Electron energy was limited to 4 Mev in order to keep 80% of the irradiation dose above 2 cm depth, the main target being the dermal region of the skin.

Evolution

Six months later a relapse with infiltration of the face needed a new course of radiotherapy. Another 5 months later chemotherapy was started with doxorubicine and with liposomal doxorubicine without results. The computerized tomography showed progression of the angiosarcoma of the head and neck with increased skin thickness and invasion of the platysma as well as multiple lymphadenopathies. After a partial regression with gemzar the disease progressed. The patient died 18 months after the diagnosis.

Comment

Cutaneous angiosarcoma of the face and scalp is a distinct entity among the angiosarcomas. It was first described in detail by Wilson Jones in 1964 [1]. This aggressive vascular neoplasm affects predominantly elderly patients with an average age of 70 years. Men are twice as frequently affected as women. The tumor is mostly localised on the scalp and the upper half of the face. There are no predisposing factors.

The clinical aspect is variable : ill-defined, bruise-like areas or facial edema with minimal erythema are the initial signs. Progressively more indurated plaques appear with nodular or ulcerated components. The neoplasm spreads quickly, centrifugally and transdermally. Multifocality is possible. Histopathologically, there is a variable degree of differentiation [2]: one distinguishes a well differentiated angiomatous pattern with irregular dissecting vascular channels in the dermis lined by pleomorphic endothelium, and an undifferentiated solid or sarcomatous pattern with a solid proliferation of polygonal endothelial cells with mitotic activity and loss of vascular spaces. Possible immunohistochemical markers for angiosarcoma include: UEA1 lectin (sensitive marker but use in conjunction with EMA and cytokeratin to exclude epithelial tumor), F VIII R Ag (highly specific, low sensitivity), CD34 (highly sensitive, stains also dermal dendrocytes, sweat gland basement membrane and haematopoietic progenitor cells) and CD31 (highly sensitive, good specificity).

The prognosis of angiosarcoma of the face and scalp is poor. The 5-year survival rate is about 12% [4]. There are no prognostic correlations with sex of the patient, clinical features or histological differentiation.

The therapy of choice is complete early surgical removal followed by electronbeam radiation therapy. Frequently the extension of the lesions renders surgery impossible, leaving only radiation therapy as an alternative treatment. In cases of metastasis polychemotherapy can be used. Unfortunately favourable responses are very rare.

Article accepted on 24/2/00

REFERENCES

1. Requena L, Sangueza O. Cutaneous vascular proliferations. Part III. J Am Acad Dermatol 1998; 38: 143-75.

2. Haustein U. Angiosarcoma of the face and scalp. Int J Dermatol 1991; 30: 851-6.

3. Poblet E, Gonzalez-Palacios F, Jimenze FJ. Different immunoreactivity of endothelial markers in well and poorly differentiated areas of angiosarcomas. Virchows Arch 1996; 428: 217-21.

4. Morrison M, Byers R, Garden A, Evans H, Ang K, Peters L. Cutaneous angiosarcoma of the head and neck. Cancer 1995; 76: 319-27.