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What is Peritoneal Mesothelioma?
Peritoneal mesothelioma is a malignancy that originates in the lining of the peritoneum, the thin membrane that bounds the abdominal and pelvic cavities. Though rare and difficult to diagnose, aggressive treatment has been shown to extend life and quality of life in peritoneal mesothelioma patients.
Malignant mesothelioma is a rare, aggressive form of cancer that develops in the mesothelial cells that form the lining of the pleura (lungs), peritoneum (abdominal cavity), pericardium (heart), and testicles (testes).
Peritoneal mesothelioma is the second most common type of mesothelioma and forms in the lining of the abdominal cavity. Only about 500 cases of peritoneal mesothelioma are diagnosed annually in the United States accounting for 15-30% of all mesotheliomas. [see References: 1,2] The average survival for patients with peritoneal mesothelioma is approximately one year; however, this can extend to 3-5 years with aggressive treatment.[3-9]
Peritoneal mesothelioma is caused by exposure to asbestos, a fibrous material woven into fabrics for fire-resistant and insulating materials. There are two suggested routes of asbestos exposure into the abdominal cavity: 1) through ingestion and 2) inhaled and transferred through the lymphatic system. Peritoneal mesothelioma is slow-growing and can take up to 10-50 years from exposure for symptoms to develop.[10]
Risk Factors
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Asbestos Exposure: Working at or living near an asbestos mine or processing plant; Working in high-risk occupational setting, such as automotive industry; Serving on military ships and facilities built with asbestos; Disturbing old asbestos products during home renovation
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Gender: occurs more commonly in men
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Age: diagnosis occurs around age 65 and older
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Genetics: associated with a rare BAP1 mutation
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Smoking: combined with asbestos exposure
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Radiation: the exact relationship is not fully understood
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Simian Virus 40: exposure to this virus may increase risk; however, the exact relationship is not fully understood
Signs & Symptoms
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Abdominal pain
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Nausea and vomiting
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Change in bowel habits
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Abdominal swelling/ascites
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Early satiety
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Unintentional weight loss
Key Facts
Asbestos
Peritoneal mesothelioma is most commonly linked to environmental exposure to asbestos.
Difficult Diagnosis
It is considered a very lethal disease that is difficult to diagnose due to vague symptoms.
Improved Treatment
Significant advances have been made in the treatment of peritoneal mesothelioma, improving both survival (median survival increased from less than 1 year to 3-5 years) and tolerability.
CRS/HIPEC
Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) is the treatment of choice for peritoneal mesothelioma, with the best outcomes achieved when all visible disease is removed.
Diagnosis and Prognosis
Malignant peritoneal mesothelioma includes three subtypes: epithelioid, sarcomatoid, and biphasic (mixed epithelioid and sarcomatoid). Epitheliod is the most common type, accounting for 50-70% of cases, and associated with the best survival outcomes. Pure sarcomatoid tumors are extremely rare, with most data limited to single case reports.[13] Regardless of subtype, complete cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) is the treatment of choice. Factors independently associated with favorable overall survival include: cancer subtype, absence of lymph node metastases, completeness of cytoreduction, and administration of HIPEC.[14]
Benign Cystic Mesothelioma & Peritoneal Inclusion Cysts
Cystic mesothelial lesions, including benign cystic mesothelioma and peritoneal inclusion cysts, pose a significant challenge for both pathologists and clinicians. While many are considered benign or reactional in nature, rather than a true neoplastic process (which forms abnormal and excessive tissue growth), the majority of patients present with multiple lesions and is associated with significant morbidity (symptoms/complications) and risk for recurrence or malignant transformation.[15] As these are very rare, with fewer than 200 cases reported in the literature, there is little information about optimal treatment. However, it is typically agreed that complete surgical resection with or without HIPEC is advisable and should be completed by specialized centers.[16]
1: Teta MJ, Mink PJ, Lau E, Sceurman BK, Foster ED. US mesothelioma patterns 1973-2002: indicators of change and insights into background rates. Eur J Cancer Prev. 2008 Nov;17(6):525-34. doi: 10.1097/CEJ.0b013e3282f0c0a2. PMID: 18941374. 2: Ahmed I, Ahmed Tipu S, Ishtiaq S. Malignant mesothelioma. Pak J Med Sci. 2013 Nov;29(6):1433-8. doi: 10.12669/pjms.296.3938. PMID: 24550969; PMCID: PMC3905399. 3: Kaya H, Sezgı C, Tanrıkulu AC, Taylan M, Abakay O, Sen HS, Abakay A, Kucukoner M, Kapan M. Prognostic factors influencing survival in 35 patients with malignant peritoneal mesothelioma. Neoplasma. 2014;61(4):433-8. doi: 10.4149/neo_2014_053. PMID: 24645844. 4: Alexander HR Jr, Bartlett DL, Pingpank JF, Libutti SK, Royal R, Hughes MS, Holtzman M, Hanna N, Turner K, Beresneva T, Zhu Y. Treatment factors associated with long-term survival after cytoreductive surgery and regional chemotherapy for patients with malignant peritoneal mesothelioma. Surgery. 2013 Jun;153(6):779-86. doi: 10.1016/j.surg.2013.01.001. Epub 2013 Mar 13. PMID: 23489943; PMCID: PMC3734959. 5: Helm JH, Miura JT, Glenn JA, Marcus RK, Larrieux G, Jayakrishnan TT, Donahue AE, Gamblin TC, Turaga KK, Johnston FM. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for malignant peritoneal mesothelioma: a systematic review and meta-analysis. Ann Surg Oncol. 2015 May;22(5):1686-93. doi: 10.1245/s10434-014-3978-x. Epub 2014 Aug 15. PMID: 25124472. 6: Baratti D, Kusamura S, Cabras AD, Bertulli R, Hutanu I, Deraco M. Diffuse malignant peritoneal mesothelioma: long-term survival with complete cytoreductive surgery followed by hyperthermic intraperitoneal chemotherapy (HIPEC). Eur J Cancer. 2013 Oct;49(15):3140-8. doi: 10.1016/j.ejca.2013.05.027. Epub 2013 Jul 4. PMID: 23831335. 7: Deraco M, Baratti D, Hutanu I, Bertuli R, Kusamura S. The role of perioperative systemic chemotherapy in diffuse malignant peritoneal mesothelioma patients treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Ann Surg Oncol. 2013 Apr;20(4):1093-100. doi: 10.1245/s10434-012-2845-x. Epub 2013 Mar 2. PMID: 23456386. 8: Kepenekian V, Elias D, Passot G, Mery E, Goere D, Delroeux D, Quenet F, Ferron G, Pezet D, Guilloit JM, Meeus P, Pocard M, Bereder JM, Abboud K, Arvieux C, Brigand C, Marchal F, Classe JM, Lorimier G, De Chaisemartin C, Guyon F, Mariani P, Ortega-Deballon P, Isaac S, Maurice C, Gilly FN, Glehen O; French Network for Rare Peritoneal Malignancies (RENAPE). Diffuse malignant peritoneal mesothelioma: Evaluation of systemic chemotherapy with comprehensive treatment through the RENAPE Database: Multi-Institutional Retrospective Study. Eur J Cancer. 2016 Sep;65:69-79. doi: 10.1016/j.ejca.2016.06.002. Epub 2016 Jul 26. PMID: 27472649. 19: Jänne PA, Wozniak AJ, Belani CP, Keohan ML, Ross HJ, Polikoff JA, Mintzer DM, Taylor L, Ashland J, Ye Z, Monberg MJ, Obasaju CK. Open-label study of pemetrexed alone or in combination with cisplatin for the treatment of patients with peritoneal mesothelioma: outcomes of an expanded access program. Clin Lung Cancer. 2005 Jul;7(1):40-6. doi: 10.3816/CLC.2005.n.020. PMID: 16098243. 10: Kim J, Bhagwandin S, Labow DM. Malignant peritoneal mesothelioma: a review. Ann Transl Med. 2017 Jun;5(11):236. doi: 10.21037/atm.2017.03.96. PMID: 28706904; PMCID: PMC5497105. 11: Plaus WJ. Peritoneal mesothelioma. Arch Surg. 1988 Jun;123(6):763-6. doi: 10.1001/archsurg.1988.01400300109019. PMID: 2453184. 12: Brigand C, Monneuse O, Mohamed F, Sayag-Beaujard AC, Isaac S, Gilly FN, Glehen O. Peritoneal mesothelioma treated by cytoreductive surgery and intraperitoneal hyperthermic chemotherapy: results of a prospective study. Ann Surg Oncol. 2006. 13: Bridda A, Padoan I, Mencarelli R, Frego M. Peritoneal mesothelioma: a review. MedGenMed. 2007 May 10;9(2):32. PMID: 17955087; PMCID: PMC1994863. 14: Yan TD, Deraco M, Baratti D, Kusamura S, Elias D, Glehen O, Gilly FN, Levine EA, Shen P, Mohamed F, Moran BJ, Morris DL, Chua TC, Piso P, Sugarbaker PH. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for malignant peritoneal mesothelioma: multi-institutional experience. J Clin Oncol. 2009 Dec 20;27(36):6237-42. doi: 10.1200/JCO.2009.23.9640. Epub 2009 Nov 16. PMID: 19917862. 15: Karpathiou G, Casteillo F, Dridi M, Peoc'h M. Mesothelial Cysts. Am J Clin Pathol. 2021 May 18;155(6):853-862. doi: 10.1093/ajcp/aqaa189. PMID: 33258870. 16: Salem Y, Farhan A, Aljawder HS, Abualsel A. Benign cystic mesothelioma: A case report on the presentation of an unusual tumor. Int J Surg Case Rep. 2021 May;82:105918. doi: 10.1016/j.ijscr.2021.105918. Epub 2021 Apr 30. PMID: 33964719; PMCID: PMC8114121. 17: Hiltbrunner S, Fleischmann Z, Sokol ES, Zoche M, Felley-Bosco E, Curioni-Fontecedro A. Genomic landscape of pleural and peritoneal mesothelioma tumours. Br J Cancer. 2022 Nov;127(11):1997-2005. doi: 10.1038/s41416-022-01979-0. Epub 2022 Sep 22. PMID: 36138075; PMCID: PMC9681755.