Malignant peritoneal mesothelioma: clinical aspects, and therapeutic perspectives

Our plain-English summary of Malignant peritoneal mesothelioma: clinical aspects, and therapeutic perspectives, by S. Boussios, M. Moschetta, A. Karathanasi, et al (Annals of Gastroenterology, 2018).

The study

• Authors: S. Boussios, M. Moschetta, A. Karathanasi, A.K. Tsiouris, F.S. Kanellos, K. Tatsi, K.H. Katsanos, D.K. Christodoulou
• Journal: Annals of Gastroenterology
• Published: 2018;31(6):659-669
• Type of article: Review
• Cited by: 53 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Malignant peritoneal mesothelioma (MPM) is a rare disease with a wide clinical spectrum. It arises from the peritoneal lining and commonly presents with diffuse, extensive spread throughout the abdomen and, more rarely, metastatic spread beyond the abdominal cavity. Computed tomography, magnetic resonance imaging and positron-emission tomography are important diagnostic tools used for the preoperative staging of MPM. The definitive diagnosis is based on histopathological analysis, mainly via immunohistochemistry. In this regard, paired-box gene 8 negativity represents a useful diagnostic biomarker for differentiating MPM from ovarian carcinoma. In addition, BRCA1-associated protein-1 (BAP1) loss is specific to MPM and allows it to be distinguished from both benign mesothelial lesions and ovarian serous tumors. Cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) has become an increasingly important therapeutic approach, while systemic therapies are still being developed. Histology, Ki-67, completeness of cytoreduction, age, sex, and baseline thrombocytosis are commonly used to optimize patient selection for CRS with HIPEC. Additionally, it is well recognized that, compared to other subtypes, an epithelial morphology is associated with a favorable prognosis, whereas baseline thrombocytosis predicts an aggressive biologicalbehavior. Platelets and other immunologic cytokines have been evaluated as potential novel therapeutic targets. Epigenetic modifiers, including BAP1, SETD2 and DDX3X, are crucial in mesothelial tumorigenesis and provide opportunities for targeted treatment. Overexpression of the closely interacting phosphoinositide 3-kinase (PI3K) and the mammalian target of rapamycin (mTOR) pathways appears crucial in regulation of the malignant phenotype. The use of targeted therapies with PI3K-mTOR-based inhibitors requires further clinical assessment as a novel approach.

In plain English

This was a review article, not a new study of patients. The authors searched PubMed for publications from September 1999 to February 2018 on malignant peritoneal mesothelioma, a cancer of the abdominal lining. They summarized its causes, symptoms, diagnosis, genetics, treatment and outlook. Citing earlier work, they described asbestos as the main cause, while noting that only 33-50% of patients report prior asbestos exposure. Talc, mica, erionite and thorotrast were also mentioned. One reviewed treatment study, a multi-institutional analysis described as covering 405 patients (the article's table lists 401), reported a median survival of 53 months and 47% survival at five years. Those patients were described as treated with surgery to remove visible tumor plus heated abdominal chemotherapy (CRS-HIPEC), though the review says the heated chemotherapy was given to 92%. The review also described tissue tests, such as BAP1 protein loss, that help distinguish this cancer from other tumors. The authors concluded that the disease remains rare, with limited treatment options and a poor outcome. They wrote that standardizing treatment will require international collaboration and prospective clinical trials.

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
• Who was studied: Publications found by a PubMed search, published between September 1999 and February 2018, on malignant peritoneal mesothelioma (and related search terms including pleural mesothelioma). Case series of patients with malignant peritoneal mesothelioma were also included. No new patients were studied.
• Exposure: Asbestos is described, citing earlier work, as the main carcinogen. Other factors mentioned are talc, mica, erionite (volcanic ash), thorotrast, Hodgkin's disease, chronic peritonitis and therapeutic radiation. No fiber types are specified.
• Main results:
  • Citing earlier work, the review states that only 33-50% of patients diagnosed with malignant peritoneal mesothelioma report prior asbestos exposure. Elsewhere it gives 33%, a lower rate than in pleural mesothelioma patients.
  • The text describes a cited multi-institutional retrospective analysis of 405 patients with malignant peritoneal mesothelioma treated with CRS-HIPEC; Table 2 lists the same study (Yan et al, 2009) with 401 patients.
  • In that analysis, HIPEC (mainly with cisplatin and doxorubicin) was given to 92% of participants.
  • In that analysis, median overall survival was 53 months and 5-year survival was 47%.
  • A cited meta-analysis of 20 studies (1047 patients treated with CRS-HIPEC) reported 5-year survival of 42% in the 67% of patients who had complete or near-complete cytoreduction.
  • According to cited work, BAP1 protein is lost in almost 50% of pleural mesotheliomas and two thirds of peritoneal mesotheliomas, but in less than 1% of high-grade serous carcinomas.
  • In cited studies of systemic chemotherapy, estimated median overall survival was 8.7 months for pemetrexed alone and 13.1 months for pemetrexed with cisplatin.
  • In one cited study, patients with sarcomatoid and biphasic tumors had a median overall survival after CRS-HIPEC of 10.5 months, compared with 51.5 months for those with more favorable histology.
• The authors' conclusion: The authors concluded that malignant peritoneal mesothelioma remains a rare disease with limited therapeutic options and a poor outcome. They said management requires careful patient selection and use of CRS and HIPEC in experienced centers, and that standardization of treatment will only come through international collaboration and prospective clinical trials.
• Limitations the authors state:
  • Because the disease is rare, the treatment evidence reviewed comes mostly from single-institution retrospective reports.
  • No randomized clinical trials have compared the available HIPEC chemotherapy regimens.
  • Prognostic factors have not been clearly identified, because of the disease's rarity and variable treatment approaches.

Topics

Medical terms in this study, explained in our Lexicon: Peritoneal Mesothelioma, Staging, Immunohistochemistry, Biomarker, Carcinoma, BAP1, Cytoreductive Surgery, HIPEC.

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