Multicystic and diffuse malignant peritoneal mesothelioma in children

Abstract

Background: Malignant and multicystic peritoneal mesotheliomas are extremely rare tumors in children, developing from mesothelial cells. No specific guidelines are available at this age. Methods: We performed a retrospective analysis of all identified children (< 18-year-old) treated in France from 1987 to 2017 for a diffuse malignant peritoneal mesothelioma (DMPM) or a multicystic peritoneal mesothelioma (MCPM). Results: Fourteen patients (5 males and nine females), aged 2.2 to 17.5 years, were included. The most frequent presenting symptoms were abdominal pain, ascitis, and alteration in the general condition. Eight patients had epithelioid mesothelioma, three had biphasic mesothelioma, and three had MCPM. Eight patients with DMPM diagnosis received cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC). Among them, six patients had neoadjuvant systemic chemotherapy, one patient, post-operative chemotherapy, and one patient CRS and HIPEC only. Three patients received only systemic chemotherapy. All patients with MCPM had only surgery. After a median follow-up of seven years (2-15), six patients (6/11; one death) with DMPM and two patients (two/three) with MCPM had a local and distant recurrences. Conclusion: Peritoneal mesothelioma in children is a rare condition with difficult diagnosis and high risk of recurrence. Worldwide interdisciplinary collaboration and networking are mandatory to help diagnosis and provide harmonious treatment guidelines.

Multicystic peritoneal mesothelioma treated with cytoreductive surgery followed or not by hyperthermic intraperitoneal chemotherapy: results from a large multicentric cohort

“…progression or of symptoms onset with HIPEC as an option [4,25]. For children with MCPM, a retrospective report suggested to reserve HIPEC as a salvage treatment in case of CRS failure [33]. The level of evidence is low and the two strategies are defensible.…”

Nivolumab for pediatric malignant peritoneal mesothelioma

“…The use of combination of cytoreductive surgery (CRS), hyperthermic intraperitoneal chemotherapy (HIPEC), and conventional chemotherapy has been reported with favorable outcomes. 3,5,6 Recently, the introduction of immune checkpoint inhibitors (ICI) in the therapeutic strategy of DMPM has shed a new light of hope for this orphan disease.…”

Pediatric Peritoneal Epithelial Malignant Mesothelioma Case Report

“…Peritoneal mesothelioma is divided into various subtypes based on clinicopathologic characteristics and consists of malignant mesothelioma, cystic mesothelioma, and well-differentiated papillary mesothelioma. Within the pediatric population, peritoneal epithelial malignant mesothelioma (PEMM) occurs more commonly in females and, unlike the adult population, there is no clear association to asbestos or radiation [ 1 , 2 ]. Ultrasonography (US) is useful in the evaluation and therapeutic drainage of ascites.…”

“…There is insufficient data on the optimal treatment strategy for managing pediatric peritoneal mesothelioma. The use of combination of neoadjuvant chemotherapy, cytoreductive surgery (CRS), and HIPEC has been reported with favorable outcomes [ 2 ].…”