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Alveolar Soft Part Sarcoma of the Nasolabial Fold: A Case Report and Literature Review

Alveolar soft part sarcoma (ASPS) is a rare malignancy that is morphologically characterized by a distinctive nodular, organoid, or nested growth pattern in which the cells are separated by vascularized septa. The diagnosis is based on a combination of pathologic and immunohistochemical findings and...

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Detalles Bibliográficos
Autores principales: Palmer, Tristan M, Yu, Westin M, Moenster, Jamie M
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10226833/
https://www.ncbi.nlm.nih.gov/pubmed/37261154
http://dx.doi.org/10.7759/cureus.38310
Descripción
Sumario:Alveolar soft part sarcoma (ASPS) is a rare malignancy that is morphologically characterized by a distinctive nodular, organoid, or nested growth pattern in which the cells are separated by vascularized septa. The diagnosis is based on a combination of pathologic and immunohistochemical findings and the presence of an ASPSCR1-TFE3 gene fusion revealed by next-generation sequencing. ASPS most commonly occurs as a painless mass in the lower extremity, with likely involvement in the lungs if metastasis is present. Here we report a case of ASPS that exhibited the characteristic ASPSCR1-TFE3 gene fusion along with a reciprocal fusion of TFE3-ASPSCR1, which presented in the nasolabial fold of a 31-year-old female. An intraoral approach was utilized for complete surgical resection of the malignancy, resulting in continued remission after 11 months.