Extraordinary Case ReportAlveolar Soft-Part Sarcoma of the TongueAlegría-Landa, Victoria MD*; Lora, Viviana MD*,†; Cota, Carlo MD†; Kutzner, Heinz MD‡; Requena, Luis MD*Author Information *Departments of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain; †Department of Dermatology, San Gallicano Dermatologic Institute for Research and Care, Rome, Italy; and ‡Dermatopathologie Laboratory, Friedrichshafen, Germany. Correspondence: Luis Requena, MD, Department of Dermatology, Fundación Jiménez Díaz, Avenida Reyes Católicos 2, 28040 Madrid, Spain (e-mail: [email protected]). V. Alegría-Landa and V. Lora have contributed equally in the performance of this article. The authors declare no conflicts of interest. The American Journal of Dermatopathology: March 2019 - Volume 41 - Issue 3 - p 218-220 doi: 10.1097/DAD.0000000000001257 Buy Metrics Abstract Alveolar soft-part sarcoma is a rare neoplasm of unknown histogenesis that accounts for less than 1% of all soft-tissue sarcomas. The tumor is highly vascularized with small vascular spaces separating nests of cells, and from cytogenetic point of view, is characterized by chromosome rearrangement der(17)t(X:17)(p11:q25) that results in the ASPL-TFE3 translocation. It can occur at any age, but it is most common between 15 and 35 years of age. The prognosis is poor, despite the relatively slow growth of the tumor. We present here an atypical case of alveolar soft-part sarcoma in which the age of the patient, the location, and the histopathologic characteristics of the lesion represented a diagnostic challenge. Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.