Cutaneous angiosarcoma afflicting the lower aspect of the left leg in an elderly woman

An 88-year-old woman who was generally in good health was referred to the D ermatology Department in August 1997. Three years prior to admission she ha d first noticed a bluish macule on her left lower leg which had rapidly enl arged over the past 3 months. Other symptoms included intermittent bleeding and pain. On examination, there was an exophytic and partly exulcerated tu mor of approximately 9 x 7 cm in diameter, located above the left medial ma lleolus. The remainder of the lower aspect of the left leg was without path ologic findings. The patient had not undergone surgery or radiotherapy prio r to the described symptoms. Blood hematology, chemistry, immunologic and serologic parameters (includin g liver function tests) were within the normal range. The alkaline phosphat ase decreased from 211 U/L initially to 179 U/L. Magnetic resonance imaging (MRI) of the left lower leg revealed a 10 x 5 x 2 cm tumor mass invading t he subcutaneous fat, leaving muscle tissue. Ultrasound of the regional lymp h nodes, a chest X-ray, and a full-chest computed tomography (CT) scan were normal. A biopsy specimen showed a marked proliferation of cells of undiff erentiated morphology in the corium and subcutaneous tissue. Several layers of atypical endothelial cells next to large cuboid cells with pleomorphic nuclei and frequent mitoses were noted, thus facilitating clear differentia tion from Kaposi's sarcoma (Fig. 1). Immunohistochemical studies were posit ive for factor VIII-related antigen, ulex europaeus I, CD31, and CD34. An a ngiosarcoma was diagnosed. Treatment included a complete surgical excision of the tumor with a wide sa fety margin of normal tissue. The skin defect was covered with mesh-graft. At this point, the patient refused consent to adjuvant radiation therapy po stoperatively. In January 1998 and in March 1998 recurrences of the tumor were again treat ed with local excision and mesh-graft. Subsequent to multifocal recurrence in June 1998 (Fig. 2A), the patient finally accepted palliative radiotherap y. Fractionated irradiation (total dose/left lower leg: 50 Gy; single fract ion dose/left lower leg: 2 Gy; 6 MV photons) resulted in local control of t he lesion for more than 6 months. In January 1999, another local recurrence of the tumor was treated with the cw-Nd:YAG laser technique as it was not possible to conduct a second course of radiation therapy. Only 1 month late r the patient presented again with multifocal spread involving most of the left lower leg (Fig. 2B). Considering the patient's age and nursing circums tances, an above-knee amputation of the left leg was performed.