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Tumor desmoplásico de pequenas células redondas abdominal da infância: relato de caso Abdominal desmoplastic small round cell tumor of childhood: case report

By Ulysses dos Santos Torres, Mariana Cezar de Andrade Ribeiro, Antonio Soares Souza and Leila Neves Bastos Borim

Abstract

O tumor desmoplásico de pequenas células redondas (TDPCR) é uma neoplasia rara e altamente agressiva, que afeta predominantemente jovens do sexo masculino. Relata-se um caso de TDPCR em um paciente do sexo masculino, de 11 anos, com acometimento intra-abdominal marcado por volumosa massa retroperitoneal em hipocôndrio esquerdo. O estudo histológico da massa revelou presença de blocos de pequenas células tumorais redondas e azuis, envoltas por estroma desmoplásico; a análise imuno-histoquímica evidenciou positividade para desmina, WT-1 e citoceratinas. Após o diagnóstico, o paciente foi submetido a tratamento quimiorradioterápico, tendo evoluído a óbito durante o 24º mês de acompanhamento.<br>Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive neoplasm, which predominantly affects young males. We report a case of DSRCT affecting an 11-year-old male patient, with intra-abdominal involvement characterized by a large retroperitoneal mass in left hypochondrium. Histological examination of the mass showed the presence of clusters of small blue round tumor cells surrounded by a dense desmoplastic stroma. Immunohistochemical analysis disclosed a positive reaction to desmin, WT-1 and cytokeratins. After diagnosis, the patient underwent chemo radiotherapy treatment, but died at the 24th month of follow-up

Topics: Tumor desmoplásico, Sarcoma de partes moles, Infância, Desmoplastic tumor, Soft tissue sarcoma, Childhood, Pathology, RB1-214
Publisher: Sociedade Brasileira de Patologia Clínica
Year: 2010
DOI identifier: 10.1590/S1676-24442010000100010
OAI identifier: oai:doaj.org/article:1f994feccfac4058be1c33aa05847cc2
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