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Notes: An 88-year-old woman who was generally in good health was referred to the Dermatology Department in August 1997. Three years prior to admission she had first noticed a bluish macule on her left lower leg which had rapidly enlarged over the past 3 months. Other symptoms included intermittent bleeding and pain. On examination, there was an exophytic and partly exulcerated tumor of approximately 9 × 7 cm in diameter, located above the left medial malleolus. The remainder of the lower aspect of the left leg was without pathologic findings. The patient had not undergone surgery or radiotherapy prior to the described symptoms.Blood hematology, chemistry, immunologic and serologic parameters (including liver function tests) were within the normal range. The alkaline phosphatase decreased from 211 U/L initially to 179 U/L. Magnetic resonance imaging (MRI) of the left lower leg revealed a 10 × 5 × 2 cm tumor mass invading the subcutaneous fat, leaving muscle tissue. Ultrasound of the regional lymph 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 undifferentiated 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 differentiation from Kaposi’s sarcoma ( 〈link href="#f1"〉Fig. 1). Immunohistochemical studies were positive for factor VIII-related antigen, ulex europaeus I, CD31, and CD34. An angiosarcoma was diagnosed.〈figure xml:id="f1"〉1 A〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD981-1:IJD_981-1_f1"/〉biopsy specimen showing marked proliferation of atypical endothelial cells in the corium and subcutaneous tissueTreatment included a complete surgical excision of the tumor with a wide safety margin of normal tissue. The skin defect was covered with mesh-graft. At this point, the patient refused consent to adjuvant radiation therapy postoperatively.In January 1998 and in March 1998 recurrences of the tumor were again treated with local excision and mesh-graft. Subsequent to multifocal recurrence in June 1998 ( 〈link href="#f2"〉Fig. 2A), the patient finally accepted palliative radiotherapy. Fractionated irradiation (total dose/left lower leg: 50 Gy; single fraction dose/left lower leg: 2 Gy; 6 MV photons) resulted in local control of the 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 later the patient presented again with multifocal spread involving most of the left lower leg ( 〈link href="#f2"〉Fig. 2B). Considering the patient's age and nursing circumstances, an above-knee amputation of the left leg was performed.〈figure xml:id="f2"〉2(A)〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD981-1:IJD_981-1_f2"/〉Multifocal recurrence in June 1998. (B) Progression of the disease with ulceration in February 1999