Recurrent Distal Epithelioid Sarcoma with Post-auricular Metastatic Nodule in a Young Adult Female

Abstract

Epithelioid sarcoma (ES) is a rare slow-growing aggressive tumor of uncertain histogenesis that preferentially affects young adult males with the mean age of 27 years at presentation. A19-year-old female represented to our hospital with 3 months history of slow-growing left posterior auricular nodule. She first presented 3 years earlier with 8 years history of a circumscribed regrowing painless right‑hand mass located between the middle and ring fingers which had previously been excised twice at a private hospital without the histopathological diagnosis. The mass at presentation measured 8 cm × 8 cm in diameter and was covered by an intact skin with a healed surgical scar. A tru‑cut biopsy was diagnosed ES-based on the cytomorphology of malignant epithelioid cells arrayed in nests, vague alveolar pattern with foci of pseudoangiomatous growth. Tumor was CD34 + focally. She had four courses of chemotherapy and tumor en bloc excision with the excision of the 3rd and 4th hand-digit with hand reconstruction. Tissue microscopy showed ES with the resection margins involvement and was CD34+, Vimentin+, epithelial membrane antigen+, S100−, and Desmin−. She did well for a year until the development of the posterior auricular nodule which was morphologically similar to the hand mass. Chest X-ray and hand radiograph revealed no lesions. ES is a distinct clinicopathologic tumor fraught with recurrences as seen in this female whose age of occurrence appears to be 9 years anecdotally. ES may be misdiagnosed due to nonspecific clinical symptoms, radiological, and pathological findings. Distant metastasis often involves lymph nodes, lungs, scalp, and bone, though the posterior auricular location was seen in this case. Large tumor size, recurrences, inadequate excision, and cutaneous metastasis are bad prognostic factors despite the young age and female sex of our patient.