Wilms' tumor is childhood's most common renal tumor, and its presentation in the adult age is extremely rare. Due to the low frequency in adults, no standard management guidelines are available for this population, also the natural history of the disease and management is unclear. We present a case report of a 31-year-old woman with metastatic Wilms' tumor, with lymph node, lung and liver involvement; systemic treatment with chemotherapy was started, with complete clinical response. Finally, a literature review is performed to showcase the differences in the clinical course, prognosis, and treatment alternatives, in adult disease compared to childhood.

Bonilla et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

El tumor desmoplásico de célula redonda y pequeña (TDCRP) es una patología neoplásica maligna agresiva y poco común. Afecta predominantemente a hombres entre la segunda y tercera década de la vida. Los pacientes que la padecen tienen un pobre pronóstico, con una supervivencia global a 5 años de hasta el 30%. Se presenta generalmente como una masa en la cavidad abdominal, frecuentemente multifocal. Para su tratamiento se recomienda un enfoque multimodal, con cirugía, quimioterapia y radioterapia. Poco más de 20 casos de TDCRP a nivel testicular/paratesticular se han reportado en la literatura. A continuación, presentamos un caso ilustrativo en ésta localización, se discute el caso y se realiza revisión de la literatura.