Diagnostic and Therapeutic Pathway in Diffuse Malignant Peritoneal Mesothelioma

Our plain-English summary of Diagnostic and Therapeutic Pathway in Diffuse Malignant Peritoneal Mesothelioma, by S. Kusamura, D. Baratti, M. De Simone, et al (Cancers, 2023).

The study

• Authors: S. Kusamura, D. Baratti, M. De Simone, E.M. Pasqual, L. Ansaloni, D. Marrelli, M. Robella, F. Accarpio, M. Valle, S. Scaringi, D. Biacchi, C. Palopoli, S. Gazzanelli, M. Guaglio, M. Deraco
• Journal: Cancers
• Published: 2023;15(3)
• Type of article: Review
• Cited by: 23 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

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).

In plain English

This article was a narrative review of how diffuse malignant peritoneal mesothelioma (DMPM), a rare cancer of the lining of the abdomen, is diagnosed and treated. The authors work at cancer centres and hospitals in Italy. The review summarized published studies and did not report new patient data. Citing other work, the authors wrote that DMPM is less frequently associated with asbestos exposure than pleural mesothelioma, and that only about 33–50% of patients with DMPM report any known prior exposure to asbestos. They cited a population-based study from the SEER database that estimated 5-year overall survival at 20% (plus or minus 5.9). They described cytoreductive surgery with hyperthermic (heated) intraperitoneal chemotherapy (HIPEC) as the current standard of care for selected patients. Across the studies they cited, median overall survival after this operation ranged from 34 to 92 months. They also reviewed tools for choosing patients for surgery, chemotherapy such as platinum drugs combined with pemetrexed, immunotherapy, and follow-up. The authors concluded that surgery with HIPEC remains the cornerstone of treatment, that its success depends on proper patient selection, and that DMPM should be managed in specialized referral centres.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Narrative review of published literature on diagnosis and treatment of diffuse malignant peritoneal mesothelioma
• Who was studied: No new patients were studied. The review covered published studies of patients with diffuse malignant peritoneal mesothelioma (DMPM), which the authors describe as about 15% of all mesotheliomas.
• Exposure: The review does not study an exposure. Citing other studies, it states that DMPM is less frequently associated with asbestos exposure than pleural mesothelioma. Its main subject is treatment: cytoreductive surgery with HIPEC, systemic chemotherapy and immunotherapy.
• Main results:
  • Citing other studies, the review states that only about 33–50% of patients diagnosed with DMPM report any known prior exposure to asbestos.
  • Citing a population-based SEER database study, the review reports that among patients with DMPM, 1-year overall survival was estimated at 46% (plus or minus 1.3) and 5-year overall survival at 20% (plus or minus 5.9).
  • In cited studies of selected DMPM patients treated with cytoreductive surgery and HIPEC, median overall survival after the operation ranged from 34 to 92 months.
  • Citing a decision-tree model based on the Ki-67 proliferation index and the extent of disease before surgery (PCI), the review reports median overall survival in three subgroups. It was 86.6 months for Ki-67 of 9% or less, 63.2 months for Ki-67 over 9% with PCI of 17 or less, and 10.3 months for Ki-67 over 9% with PCI over 17.
  • The review states that BAP1 mutation has been found in about 60% of DMPM, and that its prognostic significance remains unclear.
  • The review states that the most common chemotherapy for patients who are not candidates for surgery, or whose disease recurs, is a platinum compound combined with pemetrexed.
• The authors' conclusion: The authors concluded that cytoreductive surgery and HIPEC remain the cornerstone of treatment, and that their success depends on proper patient selection. They added that DMPM should be managed in specialized referral centres because the disease is so rare.
• Limitations the authors state:
  • The authors note that the literature lacks data on how well imaging performs in assessing DMPM.
  • For the observational studies showing a survival benefit of HIPEC, the authors state that selection bias cannot be ruled out despite multivariate analysis.
  • The authors note that clinical studies testing immune checkpoint inhibitors specifically in DMPM are usually of a very low level of evidence.

Topics

Medical terms in this study, explained in our Lexicon: Peritoneal Mesothelioma, Mesothelioma, Prognosis, Overall Survival, Cytoreductive Surgery, HIPEC, Chemotherapy, Pemetrexed (Alimta).

Related studies

• FASN for diffuse malignant peritoneal mesothelioma: a prognostic biomarker after CRS+HIPEC and a therapeutic target — V. Doldi, C.M. Ciniselli, et al. (2026)
• Splicing modulation as novel therapeutic strategy against diffuse malignant peritoneal mesothelioma — R. Sciarrillo, A. Wojtuszkiewicz, et al. (2019)
• Mesothelin and osteopontin as circulating markers of diffuse malignant peritoneal mesothelioma: A preliminary study — F. Bruno, D. Baratti, et al. (2018)
• Molecular Signatures for Combined Targeted Treatments in Diffuse Malignant Peritoneal Mesothelioma — A. Belfiore, A. Busico, et al. (2019)
• The "don't eat me" signal CD47 is a novel diagnostic biomarker and potential therapeutic target for diffuse malignant mesothelioma — C.M. Schürch, S. Forster, et al. (2017)
• The Role of Ki-67 and Pre-cytoreduction Parameters in Selecting Diffuse Malignant Peritoneal Mesothelioma (DMPM) Patients for Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) — S. Kusamura, P.A. Torres Mesa, et al. (2016)

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