RESUMEN
Synovial sarcoma (SS) accounts for 5%-10% of all soft tissue sarcomas. It is a well-defined soft tissue neoplasm with biphasic and monophasic histologic subtypes and unknown histogenesis. It usually occurs in the extremities, especially the thigh-knee region of young adults. Recurrences are frequent and distant metastasis developed in approximately half of the patients. SSs are characterized by a recurrent nonrandom chromosomal translocation, t(X; 18) (p11; q11), which is considered the primary genetic event in more than 90% of cases. Only 4 cases of cutaneous and subcutaneous SSs have been published in the literature so far. We report a case of primary subcutaneous SS in the forearm of a young woman and discuss the histopathologic differential diagnosis with other similar neoplasms. This is the first reported case of primary cutaneous SS showing immunoreactivity for TLE1 in the nuclei of neoplastic cells, supporting the use of this marker for diagnosis of this rare cutaneous neoplasm.
Asunto(s)
Biomarcadores de Tumor/inmunología , Inmunohistoquímica , Proteínas Represoras/inmunología , Sarcoma Sinovial/inmunología , Neoplasias de los Tejidos Blandos/inmunología , Tejido Subcutáneo/inmunología , Adulto , Biomarcadores de Tumor/genética , Biopsia , Proteínas Co-Represoras , Diagnóstico Diferencial , Femenino , Antebrazo , Reordenamiento Génico , Humanos , Hibridación Fluorescente in Situ , Valor Predictivo de las Pruebas , Proteínas Proto-Oncogénicas/genética , Proteínas Represoras/genética , Sarcoma Sinovial/genética , Sarcoma Sinovial/patología , Sarcoma Sinovial/cirugía , Neoplasias de los Tejidos Blandos/genética , Neoplasias de los Tejidos Blandos/patología , Neoplasias de los Tejidos Blandos/cirugía , Tejido Subcutáneo/patología , Tejido Subcutáneo/cirugíaRESUMEN
Post-transplant smooth muscle tumors (PTSMTs) are a rare and recently recognized neoplasm associated with Epstein-Barr virus (EBV). We describe the clinicopathologic, immunohistochemical and molecular features of a new case of EBV-associated PTSMT arising in the liver of a 55-year-old lung transplant recipient for lymphangioleiomyomatosis. To our knowledge, this is the third smooth muscle tumor (the second one proved to be associated with EBV) after lung transplantation. The 2 previous cases are reviewed and the differential diagnosis is also discussed.