INTRODUCTION: Very little is known about the characteristics of mesothelial tumours in the paediatric population. In adults with malignant mesothelioma, the pemetrexed-based regimen with cytoreductive surgery (CRS) is a standard of care in limited tumours, but long-term survival is uncommon. MATERIAL AND METHODS: The European Cooperative Study Group on Pediatric Rare Tumors (EXPeRT) retrospectively reviewed children, adolescents and young adults (≤21 year) diagnosed with mesothelial tumours treated between 1987 and 2018. RESULTS: Thirty-three patients were identified, 15 male and 18 female patients. One patient's exposure to asbestos was documented. Primary tumour was mainly in the peritoneum (23 patients). Histology was multicystic mesothelioma of the peritoneum (MCM) (six patients) or malignant mesothelioma (MM) (27 patients). Among MM, the first-line treatment comprised preoperative chemotherapy (14 cases), surgery only (three cases), chemotherapy only (five cases), adjuvant chemotherapy (three cases) or palliative treatment (two cases). The response rate to cisplatin-pemetrexed was 50% (6/12 cases). CRS with hyperthermic intraperitoneal chemotherapy (CRS-HIPEC) was performed in 19 patients (upfront in three, after neoadjuvant therapy in 12, or after tumour progression in six patients, including three twice). After a median follow-up of 6.7 years (range, 0-20), five-year overall and event-free survivals were 82.3% (95% CI, confidential interval ((CI), 67.8-99.9) and 45.1% (95% CI, 28.4-71.7), respectively. All patients with MCM are alive after surgery (five patients) and CRS-HIPEC (one patient). CONCLUSIONS: Paediatric mesothelioma is exceptional and seems to be different from its adult counterpart with few asbestos exposures, more peritoneal primary, and a better outcome. The cisplatin-pemetrexed regimen showed promising efficacy. Relapses could be salvaged with active therapy including CRS-HIPEC.

Bristol Royal Hospital for Children Bristol United Kingdom

Centre National Référent MESOPATH Centre Leon Berard Lyon France

Clinic of Pediatrics Dortmund Municipal Hospital Dortmund Germany

Department of Pediatric Hematology and Oncology Félix Guyon Hospital Réunion Island France

Department of Pediatric Surgery University Hospital of Saint Etienne France

Department of Pediatrics Hematology Oncology Medical University of Gdansk Poland

Hematology Oncology Division Department of Women's and Children's Health University of Padua Padua Italy

Institut Curie PSL Research University Biometry Unit Paris France

Pediatric Hematology Oncology Department Dana Children's Hospital Sourasky Medical Center Tel Aviv Israel

Pediatric Hematology Oncology Department Hospital Universitario de Cruces Barakaldo Bizkaia Spain

Pediatric Hematology Oncology Department La Timone Hospital Marseille France; Centre de Recherche en Cancérologie de Marseille Inserm U1068 Aix Marseille Univ Marseille France

Pediatric Hematology Oncology Department Rambam Medical Center Haifa Israel

Pediatric Hematology Unit Kaplan Medical Center Rehovot Israel

Pediatric Oncology and Hematology University Children's Hospital Tuebingen Germany

Pediatric Oncology Department University Hospital Brno Masaryk Czech Republic

Pediatric Oncology Unit Fondazione IRCCS Istituto Nazionale dei Tumori Milan Italy

Pediatric Surgery Department of Women's and Children's Health University of Padua Padua Italy

SIREDO Oncology Center PSL Research University Institut Curie Paris France

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