Continuous hyperthermic peritoneal perfusion for desmoplastic small round cell tumor

Area of Science:

• Pediatric Oncology
• Surgical Oncology
• Medical Oncology

Background:

• Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive cancer primarily affecting children, adolescents, and young adults.
• DSRCT typically presents as diffuse abdominal metastatic disease on peritoneal surfaces, resembling carcinomatosis.
• Current aggressive treatment protocols for DSRCT have historically resulted in poor patient outcomes.

Purpose of the Study:

• To report the first pediatric cases of DSRCT treated with intraoperative continuous hyperthermic peritoneal perfusion (CHPP).
• To evaluate the feasibility and potential of CHPP combined with cisplatin in managing DSRCT in pediatric patients.
• To introduce a novel therapeutic strategy for a rare and challenging pediatric malignancy.

Main Methods:

• Two pediatric cases of DSRCT were treated with aggressive surgical excision.
• Intraoperative continuous hyperthermic peritoneal perfusion (CHPP) using cisplatin was administered following surgical resection.
• This represents the initial application of CHPP, typically used for adult carcinomatosis, in pediatric DSRCT cases.

Main Results:

• The study presents two pediatric case reports detailing the treatment of DSRCT.
• The treatment involved aggressive surgical excision followed by intraoperative CHPP with cisplatin.
• These cases highlight the application of a technique usually reserved for adult malignancies in a pediatric setting.

Conclusions:

• Intraoperative continuous hyperthermic peritoneal perfusion (CHPP) with cisplatin, following aggressive surgical excision, represents a novel treatment approach for pediatric DSRCT.
• This case series is the first to describe the use of CHPP in pediatric DSRCT patients.
• Further research is warranted to establish the efficacy and long-term outcomes of this treatment strategy in young patients with DSRCT.