Diagnostic and Therapeutic Pathway in Diffuse Malignant Peritoneal Mesothelioma

Abstract

Diffuse malignant peritoneal mesothelioma (DMPM) is a rare form of mesothelioma that carries a very poor prognosis. The 5-year overall survival is about 20% (±5.9). Survival is optimal for patients suitable for cytoreductive surgery (CRS) with Hyperthermic Intraperitoneal Chemotherapy (HIPEC), with a median OS ranging from 34 to 92 months. However, selecting patients for surgery remains a complex task and requires a careful preoperative workup, rational analysis of prognostic profiles, and risk prediction models. Systemic chemotherapy could be offered: (1) in the adjuvant setting for high-risk patients; (2) for patients not eligible for CRS; and (3) for those with recurrent disease. It mainly includes the combination of Platin compound with Pemetrexed or immunotherapy. The biology of DMPM is still largely unknown. However, progress has been made on some fronts, such as telomere maintenance mechanisms, deregulation of apoptosis, tyrosine kinase pathways, and mutation of BRCA1-associated protein 1 (BAP1). Future perspectives should include translational research to improve our understanding of the disease biology to identify druggable targets. We should also clear the role of immune checkpoint inhibitors and investigate new locoregional technologies, such as pressurized intraperitoneal aerosol chemotherapy (PIPAC) or normothermic intraperitoneal chemotherapy (NIPEC).

Telomere length and TERT polymorphisms as biomarkers in asbestos-related diseases

“… 19 , 33 , 34 Furthermore, cancer cells have typically short telomeres 18 , 20 , 21 , as also shown for MM. 23 However, the presence of telomerase reactivation in MM 22 allows for an unlimited cell division potential and telomere length maintenance, which, on the contrary, does not occur in non-neoplastic cells, 35 potentially contributing to the observed results.…”

De novo germline TP53 mutation in a pediatric patient with Li–Fraumeni syndrome and diffuse peritoneal mesothelioma

“…The combination of cytoreductive surgery (CRS), hyperthermic intraperitoneal chemotherapy (HIPEC), and conventional chemotherapy can be utilized; however, despite the treatment, the disease often follows a fatal course. 1 The latest strides in immunology have also reached into the domain of mesothelioma studies. 2 This presentation illuminates the case of the first pediatric patient diagnosed with DMPM, who was successfully treated with conventional chemotherapy, CRS, HIPEC, and nivolumab, while also diagnosed with Li-Fraumeni syndrome (LFS).…”

Mesothelioma: Peritoneal, Version 2.2023, NCCN Clinical Practice Guidelines in Oncology

“…11 Survival is improved for patients who are able to undergo complete cytoreductive surgery (CRS) with intraperitoneal chemotherapy. [12][13][14][15][16] There are multiple intravenous systemic therapy options for patients who are not candidates for CRS, or those whose disease recurs following CRS with or without hyperthermic intraperitoneal chemotherapy (HIPEC).…”

“…The NCCN Guidelines for Mesothelioma: Peritoneal recommend that patients with PeM should have their treatment managed by a multidisciplinary team with experience in PeM. 12,21 Treatment options for patients with diffuse PeM include surgery and/or systemic therapy. 21,74 Select patients with medically operable diffuse PeM and good performance status (PS) are candidates for multimodality therapy, including those with epithelioid histology and unicavitary disease.…”