Key result
Recurrent peritoneal desmoplastic small round cell tumor presents with rare pleural and pulmonary metastases.
Case Report (n=1)
This case report highlights a rare presentation of pleural and pulmonary metastases from recurrent peritoneal desmoplastic small round cell tumor.
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Supports multimodal therapy in DSRCT; leaves open optimal HIPEC and RT integration pending dedicated RCTs.
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.
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Mouhssine N.*, Msika S., Arfaoui H., Sqalli Houssini Z., Bamha H., Bougteb N., EL Khattabi W., Afif My. H. (2026) conducted a case report in Desmoplastic small round cell tumor (DSRCT) (n=1). A 22-year-old patient presented with rare pleural and pulmonary metastases from a recurrent peritoneal desmoplastic small round cell tumor.
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