Peritoneal mesothelioma: the site of origin matters

Our plain-English summary of Peritoneal mesothelioma: the site of origin matters, by H.L. Kindler (American Society of Clinical Oncology Educational Book, 2013).

The study

• Author: H.L. Kindler
• Journal: American Society of Clinical Oncology Educational Book
• Published: 2013:182-8
• Type of article: Review
• Cited by: 35 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

The etiology, gender distribution, pathology, natural history, and treatment options for mesothelioma (MM) differ substantially depending on the site of origin. Peritoneal mesothelioma (MPeM) is a rare disease, comprising only approximately 10% to 15% of the 2,500 cases of MM diagnosed in the United States each year. Patients with MPeM are younger than patients with pleural MM, and a higher proportion, mostly women, are long-term survivors. Most MPeM is caused by asbestos exposure. Germ-line mutations of BAP1 (BRCA associated protein 1) can predispose to MM, uveal melanoma, and potentially other cancers. MPeM can be challenging to diagnose, and cytology is rarely helpful. Review by an experienced pathologist using a panel of at least two positive and two negative immunohistochemical stains is essential. The three major pathologic subtypes are epithelial, sarcomatoid, and biphasic. Most cases are epithelial; the others have a dismal prognosis. Two indolent subtypes of borderline malignant potential-well-differentiated papillary mesothelioma and benign multicystic mesothelioma-are more common in the peritoneum and are treated surgically. In highly selected patients receiving treatment at experienced referral centers, an aggressive locoregional strategy that combines cytoreductive surgery to remove all gross disease and hyperthermic intraperitoneal chemotherapy to treat residual microscopic tumors yields a 3-year survival of 60% and a median survival approaching 5 years, far better than expected from historic controls. This approach also provides durable palliation of malignant ascites in nearly all patients. Pemetrexed is the only U.S. Food and Drug Administration (FDA)-approved systemic chemotherapy for pleural MM. Largely on the basis of data from pharmaceutical registry studies, the activity of pemetrexed-based chemotherapy appears to be similar in pleural MM and MPeM.

In plain English

This 2013 article is a narrative review written for a cancer-education book. It is not a new study. The author summarized published research on peritoneal mesothelioma, which starts in the lining of the abdomen, and compared it with pleural mesothelioma. Citing the U.S. SEER cancer database, the author reported that peritoneal patients were younger (mean age 63.3 vs. 70.8 years). Women made up 44% of peritoneal cases but only 19% of pleural cases. The review stated that most peritoneal mesothelioma is caused by asbestos exposure, that the risk attributable to asbestos is lower in women, and that inherited BAP1 mutations can predispose people to mesothelioma. A cited registry of 405 patients at eight institutions reported a median survival of 53 months, and 3- and 5-year survival of 60% and 47%. These patients were candidates for surgery to remove visible tumor plus heated chemotherapy delivered inside the abdomen. The author concluded that select patients treated at specialized centers can achieve prolonged survival, and that pemetrexed-based chemotherapy appears to work similarly in both sites. The author noted that the clinical trials are small and nonrandomized.

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 (educational book chapter) of published research on peritoneal mesothelioma; it reports no new data of its own
• Who was studied: Published studies of patients with peritoneal mesothelioma, mainly U.S. data and series from specialist referral centers, compared with pleural mesothelioma; peritoneal cases make up about 10% to 15% of roughly 2,500 U.S. mesothelioma cases per year
• Exposure: Asbestos exposure is described as the main cause. Other potential causes listed are thorotrast, erionite, therapeutic radiation, familial Mediterranean fever and other causes of chronic peritonitis. The review also covers treatments: cytoreductive surgery with hyperthermic intraperitoneal chemotherapy, and pemetrexed-based chemotherapy.
• Main results:
  • In the SEER database (cited study), patients with peritoneal mesothelioma were younger than those with pleural mesothelioma: mean age 63.3 vs. 70.8 years.
  • In the SEER database (cited study), women accounted for 19% of pleural mesothelioma cases and 44% of peritoneal mesothelioma cases.
  • The review stated that the attributable risk from asbestos exposure is lower in women.
  • In cited studies of asbestos miners and insulators, the proportion of peritoneal mesothelioma was highest among those with the greatest cumulative asbestos exposure.
  • Germ-line BAP1 mutations were described as predisposing to mesothelioma, uveal melanoma, other melanocytic tumors and potentially other cancers.
  • The review stated that untreated peritoneal mesothelioma has a median survival of about 6 months.
  • In a cited multi-institutional registry of 405 surgery candidates (eight institutions, six countries, 1989 to 2009), overall median survival was 53 months. The 3- and 5-year survival rates were 60% and 47%.
  • In that registry, women survived substantially longer than men: median 119 vs. 36 months.
  • In the cited U.S. Expanded Access Program for pemetrexed, 98 of 1,056 mesothelioma patients had peritoneal disease. Among assessable peritoneal patients, the disease control rate was 71%.
• The authors' conclusion: The author concluded that select patients treated at specialized centers with cytoreductive surgery and HIPEC can achieve prolonged survival. The author also concluded that pemetrexed-based chemotherapy appears to have similar activity in pleural and peritoneal mesothelioma, and that international collaboration is essential.
• Limitations the authors state:
  • Clinical trials in peritoneal mesothelioma are infrequent, small and nonrandomized. Treatment approaches are largely extrapolated from selected patients at referral centers.
  • The pharmaceutical expanded access program data on pemetrexed should be interpreted cautiously. Their patient groups are more heterogeneous, and their assessment and reporting are less rigorous than in prospective trials.

Topics

The National Library of Medicine indexes this study under subjects our site covers: Lung cancer, Mesothelioma, Peritoneal mesothelioma.

Medical terms in this study, explained in our Lexicon: Peritoneal Mesothelioma, Etiology, Pathology, Mesothelioma, Asbestos Exposure, BAP1, Cytology, Sarcomatoid.

Related studies

• Complete cytoreductive surgery plus HIPEC for peritoneal metastases from unusual cancer sites of origin: results from a worldwide analysis issue of the Peritoneal Surface Oncology Group International (PSOGI) — D. Goéré, G. Passot, et al. (2017)
• Peritoneal carcinomatosis from unusual cancer origins: Is there a role for hyperthermic intraperitoneal chemotherapy? — C. Honoré, D. Goéré, et al. (2016)
• Detecting germline BAP1 mutations in patients with peritoneal mesothelioma: benefits to patient and family members — M. Kittaneh, C. Berkelhammer (2018)
• Molecular Analysis of a Patient With Neurofibromatosis 2 (NF2) and Peritoneal Malignant Mesothelioma — C. Glass, L.M. Sholl, et al. (2020)
• Comparison of genomic abnormality in malignant mesothelioma by the site of origin — M. Takeda, T. Kasai, et al. (2014)
• The prognostic significance of BAP1, NF2, and CDKN2A in malignant peritoneal mesothelioma — A.D. Singhi, A.M. Krasinskas, et al. (2016)

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