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Case Reports

Abstract

Epithelioid sarcoma is an extremely rare soft tissue tumor, mainly affecting young to middle-aged adults, with a slightly higher occurrence in males. It most often occurs as a dermal or subcutaneous nodule often complicated by ulceration and necrosis of lesions that can lead to confounding suspicion for inflammatory, infectious, or granulomatous processes. Local advancement is common, marked by lymph node involvement or in-transit metastasis, while distant metastasis often involves the lung, brain, and bones. Here, we report a case of epithelioid sarcoma presenting in a sporotrichoid pattern. The patient, a 21-year-old male, initially presented with a chronic wound on the left forearm, unresponsive to various antibiotics. Biopsies disclosed a nodular proliferation in the dermis of pleomorphic round to polygonal cells palisading around a large area of necrosis, reminiscent of a caseating granuloma. Immunohistochemical studies, however, revealed no staining for CD68 and diffuse expression of vimentin and pankeratin suggesting a tumor of mesenchymal origin with epithelioid differentiation. Imaging studies revealed tumor with entrapment and tracking of the fascia in the volar and dorsal forearm compartments and suspicious left-sided axillary lymphadenopathy. Epithelioid sarcoma poses significant challenges in treatment, especially in cases of advanced or metastatic disease, with historically poor survival rates. While sporotrichoid presentations typically indicate infectious conditions, this case underscores the importance of considering rare malignancies like epithelioid sarcoma, especially when standard treatments fail. The case also highlights the need for early consideration of disease-specific therapies and careful evaluation of treatment strategies in such complex presentations.