Brief ReportEpithelioid Rhabdomyosarcoma Report of a Cutaneous Case and Literature Review of a Recently Described Variant of RhabdomyosarcomaValério, Ediel MD*; Almeida, Gisele C. MD, PhD†; Neotti, Tatiane MD*; Nascimento, Antonio G. MD*; Bezerra, Stephania M. MD*; Costa, Felipe D. MD, PhD*Author Information *Department of Pathology, A.C. Camargo Cancer Center, São Paulo, Brazil; and †Department of Pathology, Barretos Cancer Hospital, São Paulo, Brazil. Correspondence: Ediel Valério, MD, Department of Pathology, A. C. Camargo Cancer Center, Rua Professor Antonio Prudente, São Paulo, Brazil 01509-010 (e-mail: email@example.com). E. Valério wrote the manuscript. F. D. Costa, A. G. Nascimento, and G. C. Almeida conceived the idea, and edited and reviewed the manuscript. Dras. T. Neotti and S. M. Bezerra evaluated the histological findings and also reviewed the manuscript. All authors gave the final approval for publication. The authors declare no conflicts of interest. The author/coauthors declare that the study complies with ethical standards. Online date: April 29, 2020 The American Journal of Dermatopathology: April 2020 - Volume 42 - Issue 4 - p 275-279 doi: 10.1097/DAD.0000000000001560 Buy Metrics Abstract Rhabdomyosarcoma affects mainly pediatric patients and is currently classified into 4 categories: embryonal, alveolar, pleomorphic, and spindle cell/sclerosing. Epithelioid rhabdomyosarcoma is a recently described variant of rhabdomyosarcoma in which primary cutaneous presentation is infrequent. In this brief report, we describe a rare case of epithelioid rhabdomyosarcoma in an 81-year-old man, presenting as a skin lesion in the neck, which increased in size in 1 month. After imaging evaluation, a solid cervical mass was discovered. A biopsy was performed, and the diagnosis of epithelioid rhabdomyosarcoma was rendered. The patient died due to rapid progression of the tumor. To make an accurate diagnosis and ensure appropriate patient management, it is necessary to be aware of this variant and use proper immunohistochemical stains when facing an epithelioid malignancy, expanding the differential diagnosis of epithelioid neoplasms. Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.