Multicystic and diffuse malignant peritoneal mesothelioma in children

Area of Science:

• Pediatric Oncology
• Surgical Oncology
• Medical Oncology

Background:

• Malignant and multicystic peritoneal mesotheliomas are exceptionally rare pediatric tumors.
• No established treatment guidelines exist for children diagnosed with these conditions.

Purpose of the Study:

• To analyze treatment outcomes for children with diffuse malignant peritoneal mesothelioma (DMPM) and multicystic peritoneal mesothelioma (MCPM).
• To evaluate the effectiveness of cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) and systemic chemotherapy.

Main Methods:

• Retrospective analysis of 14 children (<18 years) treated in France between 1987 and 2017.
• Inclusion of patients with DMPM and MCPM.
• Review of treatment modalities including CRS, HIPEC, and systemic chemotherapy, with a median follow-up of seven years.

Main Results:

• Eight DMPM patients received CRS with HIPEC, with varying chemotherapy regimens.
• Three MCPM patients underwent surgery alone.
• Recurrence rates were high, with 6 out of 11 DMPM patients and 2 out of 3 MCPM patients experiencing local or distant recurrence.

Conclusions:

• Pediatric peritoneal mesothelioma is a rare entity with challenging diagnosis and a high risk of recurrence.
• Interdisciplinary collaboration and international networking are crucial for improving diagnosis and establishing standardized treatment guidelines.