PLEURO-PULMONARY METASTASES FROM RECURRENT PERITONEAL DESMOPLASTIC SMALL ROUND CELL TUMOR (DSRCT)

Desmoplastic small round cell tumors (DSRCT) belong to the group of malignant small round cell tumors that primarily affect children and young adults. This group includes Ewing sarcomas, peripheral neuroectodermal tumors (PNET), embryonal rhabdomyosarcomas, neuroblastomas, and desmoplastic small round cell tumors. DSRCT is a rare and highly aggressive malignant tumor. Its origin is most often intra-abdominal, without an obvious visceral primary site, and it is almost always associated with the presence of an abdominal mass and/or peritoneal carcinomatosis. More rarely, initial mediastinal, pleural, paratesticular, osseous, and intracranial localizations have been reported.[1] We report the case of a 22-year-old patient with pleural and pulmonary involvement of desmoplastic small round cell tumors.