Department of Dermatology, Mayo Clinic, Rochester, Minnesota
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A 27-year-old woman presented with a 2-year history of a progressively enlarging, painful ulcer on her right foot. Two years earlier, she had noticed an apparent wart on her right foot. The lesion had been treated with liquid nitrogen. An ulcer developed at the site of treatment and enlarged progressively, becoming so painful that she had difficulty walking. Extensive surgical debridement and closure were unsuccessful in healing the ulcer; the ulcer grew larger and more painful. After an amputation was recommended by her local doctors, the patient sought another opinion.At physical examination, the patient had a painful, 9.5 cm × 5 cm ulcer on the plantar aspect of the right foot (Fig. 1). Exuberant, rolled borders were present, and a yellow exudate covered the base of the lesion. The right inguinal lymph nodes were enlarged and firm. A punch biopsy specimen from the ulcer border was examined.Microscopic examination of a hematoxylin and eosin preparation of the punch biopsy specimen showed a reasonably well-demarcated neoplasm within the deep reticular dermis down to the dermal-pannicular junction (Fig. 2). This proliferation was composed of a population of round cells and spindle cells. The round cells were arranged in nests separated by delicate, fibrous septa, and the spindle cell proliferation was intercalated between collagen bundles. The nuclei of both cell types were uniform and vesicular with prominent nucleoli. No typical or atypical mitotic figures were identified within this proliferation. Staining with S-100 protein was strongly positive. These findings were consistent with a clear cell sarcoma.A biopsy specimen from the right inguinal lymph node was positive for metastatic clear cell sarcoma. Chest radiography and computed tomography showed multiple nodules throughout both lungs. The patient received five cycles of therapy with cisplatin, vinblastine, dacarbazine, and interferon-α, and is alive 2 years later.