Metastatic desmoplastic small round cell tumor controlled by an anthracycline-based regimen: review of the role of chemotherapy.

Desmoplastic small round cell tumors (DSRCT) are rare and aggressive malignant neoplasms that usually occur in young males. They usually present intraabdominally with widespread peritoneal involvement at diagnosis and are sometimes associated with lymphadenopathies and liver metastases. Intraabdominal DSRCT is usually disseminated; no curative outcome has yet been achieved. We report a case of metastatic intraabdominal DSRCT controlled by an anthracycline-based regimen. A literature review of the treatment options, mainly chemotherapy, available for this unusual neoplasm, will be discussed. In patients with unresectable or metastatic disease, symptom control is most important because treatment modalities minimally impact survival. Palliative chemotherapy (mainly monotherapy) is preferable.