Proximal-type epithelioid sarcoma – a rare, aggressive subtype of epithelioid sarcoma presenting as a recurrent perineal mass in a middle- aged maleReportar como inadecuado




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World Journal of Surgical Oncology

, 5:28

First Online: 06 March 2007Received: 26 December 2006Accepted: 06 March 2007

Abstract

BackgroundEpithelioid sarcoma ES is an uncommon soft tissue sarcoma. Lately, subtypes of ES, including proximal-type ES have been recognized, with relatively few reports on such cases.

Case presentationA 47-year-old male presented with a perineal soft tissue mass that was excised elsewhere and the biopsy was submitted for a review diagnosis. On histology, a multi nodular tumor was seen comprising sheets of oval to polygonal cells with moderate amount of cytoplasm. Interspersed were larger, rhabdoid cells with abundant eosinophilic cytoplasm and prominent nucleoli. Focal necrosis was noted. A wide panel of immunohistochemical IHC markers was performed to rule out a range of differential diagnoses, including a poorly differentiated carcinoma, a melanoma and a variety of sarcomas with epithelioid differentiation. On IHC, the tumor cells showed a polyphenotypic expression, including positivity for epithelial markers i.e cytokeratin CK, CK7, EMA and mesenchymal markers like vimentin and CD 34. Desmin was focally positive. CK20, CEA, S-100, HMB-45, SMA, LCA and CD31 were negative. A diagnosis of a proximal-type ES was formed. Six moths later, despite adjuvant chemo and radiotherapy CT and RT, the patient continued to have the lesion and was referred again. In addition to the earlier histological features, sections from the persistent tumor mass showed an increased number of larger cells along with multinucleated tumor giant cells.

ConclusionThe value of identifying this uncommon tumor from a list of differential diagnoses is in view of its aggressive behavior, as seen in our case. A wide excision with clear margins is imperative with options of post-operative CT-RT in individual cases during a close follow-up.

Electronic supplementary materialThe online version of this article doi:10.1186-1477-7819-5-28 contains supplementary material, which is available to authorized users.

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Autor: Bharat Rekhi - Biru D Gorad - Roshni F Chinoy

Fuente: https://link.springer.com/



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