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.