Intraabdominal and ganglionic desmoplastic small round cell tumor: a case series

Area of Science:

• Oncology
• Rare Cancers
• Sarcomas

Background:

• Desmoplastic small round cell tumor (DSRCT) is a rare malignancy with a poor prognosis, primarily affecting young males.
• DSRCT often presents as a large abdominal mass with extensive peritoneal spread at diagnosis.
• Metastatic disease can occur in advanced stages of DSRCT.

Observation:

• This study retrospectively analyzed four cases of DSRCT in young North African males.
• Cases involved intra-abdominal masses and one case with ganglionic involvement without a discernible mass.
• All cases were confirmed by the characteristic t(11;22) translocation.

Findings:

• Treatment involved a multidisciplinary approach including surgery, chemotherapy, and radiotherapy.
• Despite aggressive multimodal therapy, including high-dose chemotherapy and extensive resection, outcomes remained poor.
• One patient with ganglionic DSRCT showed no response to chemotherapy and was lost to follow-up.

Implications:

• Current treatment paradigms for DSRCT, while multidisciplinary, are largely insufficient to overcome the disease's lethality.
• The findings underscore the urgent need for developing more effective therapeutic strategies for DSRCT.
• Further research into the molecular mechanisms and novel treatment modalities for DSRCT is critical.