Um homem de 64 anos com um pacemaker para síndrome do seio doente queixou-se de polaciúria 5 meses antes de se apresentar no nosso hospital. Os resultados do exame abdominal foram normais, e não foi encontrado nenhum tumor palpável ou nódulo linfático aumentado no pescoço, abdómen, axila ou região inguinal. Não foram obtidos resultados anormais dos exames de sangue e urina. Os testes para antígeno carcinoembrionário, alfa-fetoproteína, antígeno do cancro (CA) 19-9, CA125, e marcadores de carcinoma de células escamosas foram todos normais. A ultrassonografia revelou uma massa de 10 × 8 × 6 cm de baixo eco e uma região de alto eco no abdómen inferior. Também foram observadas regiões císticas. A tomografia computorizada com contraste (CE-CT) demonstrou um tumor moderadamente lobulado, interno, heterogéneo de 10 × 8 × 7 cm com regiões hipervasculares; no entanto, não foi detetada calcificação ou componentes gordos. Observou-se a compressão da bexiga pelo tumor. A fronteira do tumor foi clara, e não foi detetada evidência de invasão direta. O CE-CT também não revelou metástases no fígado ou pulmão ou aumento de linfonodos intra-abdominais. Portanto, a cirurgia foi realizada. O tumor foi encontrado na área pélvica retroperitoneal abaixo do mesentério do cólon sigmóide. Não foi observada aderência intestinal ou invasão do tumor da bexiga ou de outros órgãos. A ressecção ocorreu sem incidentes. O tempo de operação foi de 1 h 59 min, e a perda de sangue intraoperatória foi de 241 ml. Um tumor sólido, duro e elástico de 13 × 8.0 × 5.5 cm, 420 g, foi excisado. A superfície cortada era cinza-branca e incluía um componente cístico e um foco hemorrágico. Patologicamente, o tumor continha células ovais ou fusiformes que cresciam num padrão aleatório numa matriz de colagénio (padrão sem padrão). As margens cirúrgicas foram negativas. Imuno-histoquimicamente, a amostra foi positiva para CD34, bcl-2, bem como vimentina, mas negativa para c-kit. Os testes imunoistoquímicos para p53, S-100 e alfa-actina do músculo liso foram todos negativos. Com base nestes achados, foi diagnosticada SFT retroperitoneal da pelve. O curso pós-operatório foi sem intercorrências e a polaciúria desapareceu imediatamente. A alta ocorreu no dia 10 pós-operatório. Não foi detetada qualquer recorrência 20 meses após a cirurgia. SFT é um tumor de tecido mole identificado pela primeira vez na pleura por Klempler et al. em 1931 [] É mais comum na pleura, como demonstrado por Gold et al., que relataram que 54 de 79 casos de SFT (68%) foram encontrados na pleura [] A SFT também surge na região retroperitoneal [] como no nosso caso, e foi relatado na órbita ocular [] e região intracraniana [] A SFT retropertoneal é geralmente diagnosticada pela presença de uma distensão abdominal ou de uma massa palpável [] A polaciúria tem sido associada com a SFT [] como no nosso caso, e também pode estar relacionado com dificuldade em urinar [] A análise patológica de SFT mostrou células tumorais atípicas mal diferenciadas dispostas de forma aleatória em fibras de colágeno hialinizado (padrão sem padrão) ou em um padrão de crescimento semelhante a um hemangiopericytoma [] Os achados imunohistoquímicos característicos de SFT incluem alta positividade para CD34, que é conhecido como o antígeno relacionado com fibroblastos. Os resultados positivos dos testes imunohistoquímicos para bcl-2 e vimentina e os resultados negativos para c-kit também são úteis no diagnóstico de SFT [,]. O tumor, neste caso, demonstrou essas características de SFT. O tratamento de primeira linha para SFT é a ressecção cirúrgica. Margens negativas e ressecção cirúrgica completa são essenciais para um bom prognóstico [] A inspeção completa da imagem deve ser realizada para determinar a extensão da invasão do tumor em torno dos órgãos, bem como para assegurar a ressecção cirúrgica completa e a preservação da função do órgão. Neste caso, a ressonância magnética não foi possível porque um pacemaker tinha sido previamente implantado. No entanto, a CE-CT revelou a ausência de invasão do tumor em outros órgãos, inchaço dos gânglios linfáticos e metástases distais. Por conseguinte, foi realizada a ressecção completa. A classificação de tumores de tecidos moles da Organização Mundial de Saúde em 2002 categorizou o SFT extrapleural como um tumor fibroblástico/miofibroblástico de malignidade intermédia, que é definido como um tumor mesenquimal ubíquo de possível tipo fibroblástico com um proeminente padrão vascular ramificado semelhante a um hemangiopericytoma [] As características patológicas de SFT maligno incluem lesões hipercelulares, atipia citológica, numerosas mitoses (4 ou mais mitoses por 10 campos de alta potência), necrose tumoral e/ou margens infiltrativas [("-", "O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), ("O"), 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(".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), (".", "O"), [] Eles sugeriram uma possível associação entre a mutação p53 e a transformação benigna para maligna em SFT. Portanto, a expressão de CD34, bcl-2 e p53 pode ser importante para prever o prognóstico de pacientes com SFT.