Introduction
Mesothelioma is a rare and aggressive tumor that originates from the serosal lining of the pleural, peritoneal and pericardial cavities. Although pediatric mesotheliomas are more frequently located in the pleura than in the peritoneum, the incidence of peritoneal mesotheliomas are proportionally higher in children than in adults [1].
It is often associated with asbestos exposure but prior exposure to asbestos cannot be demonstrated in all patients diagnosed with malignant mesothelioma [2-4]. Although the pathogenesis of mesothelioma has not been clearly elucidated, there are genetic and epigenetic mechanisms as well as environmental factors. Environmental and genetic factors influence the inflammatory tumor microenvironment. Two of the most frequently mutated tumor suppressor genes in mesothelioma are neurofibromatosis type 2 and BRCA1-associated protein-1 genes. Alterations in DNA methylation patterns (especially in E-cadherin, fragile histidine triad, retinoic acid receptor and wnt inhibitory factor-1 and dysregulation of microRNA expression may also contribute to the development of mesothelioma [2].
The diagnosis and treatment of with diffuse malignant peritoneal mesothelioma (DMPM) in children is challenging due to the rarity of the disease and lack of the specific guidelines. The treatment choice for mesothelioma in children is extrapolated from studies in adult patients. 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, especially in the first-line setting.
Here, we report 13-year-old girl diagnosed with DMPM patient. In the treatment of our case CRS, HIPEC, platinum and pemetrexed-based conventional chemotherapy and immune checkpoint inhibitor nivolumab were used. To our best knowledge, this is the first pediatric case report of the nivolumab combined with CRS, HIPEC and conventional chemotherapy for treatment of DMPM.