Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Presentation, initial evaluation, and prognosis of malignant pleural mesothelioma

Daniel H Sterman, MD
Leslie A Litzky, MD
Larry R Kaiser, MD
Section Editors
Rogerio C Lilenbaum, MD, FACP
Eric Vallières, MD, FRCSC
Deputy Editor
Sadhna R Vora, MD


Malignant mesothelioma is a rare and insidious neoplasm with a poor prognosis. It arises from mesothelial surfaces of the pleural cavity, peritoneal cavity, tunica vaginalis, or pericardium. Malignant pleural mesothelioma (MPM) is the most common type and can be difficult to treat because most patients have advanced disease at presentation.

The clinical presentation, evaluation, staging, and prognosis of MPM will be reviewed here. Epidemiology and treatment of pleural mesothelioma, as well as issues related to peritoneal mesothelioma are covered elsewhere:

(See "Epidemiology of malignant pleural mesothelioma".)

(See "Pathology of malignant pleural mesothelioma".)

(See "Initial management of malignant pleural mesothelioma".)

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Mar 06, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Socola F, Loaiza-Bonilla A, Bustinza-Linares E, et al. Recurrent thrombotic thrombocytopenic purpura-like syndrome as a paraneoplastic phenomenon in malignant peritoneal mesothelioma: a case report and review of the literature. Case Rep Oncol Med 2012; 2012:619348.
  2. Wong SF, Newland L, John T, White SC. Paraneoplastic leukocytoclastic vasculitis as an initial presentation of malignant pleural mesothelioma: a case report. J Med Case Rep 2012; 6:261.
  3. Li JY, Yong TY, Kuss BJ, et al. Malignant pleural mesothelioma with associated minimal change disease and acute renal failure. Ren Fail 2010; 32:1012.
  4. Bech C, Sørensen JB. Polyneuropathy in a patient with malignant pleural mesothelioma: a paraneoplastic syndrome. J Thorac Oncol 2008; 3:1359.
  5. Tanriverdi O, Meydan N, Barutca S, et al. Anti-Yo antibody-mediated paraneoplastic cerebellar degeneration in a female patient with pleural malignant mesothelioma. Jpn J Clin Oncol 2013; 43:563.
  6. Patz EF Jr, Shaffer K, Piwnica-Worms DR, et al. Malignant pleural mesothelioma: value of CT and MR imaging in predicting resectability. AJR Am J Roentgenol 1992; 159:961.
  7. Boutin C, Rey F, Gouvernet J, et al. Thoracoscopy in pleural malignant mesothelioma: a prospective study of 188 consecutive patients. Part 2: Prognosis and staging. Cancer 1993; 72:394.
  8. Husain AN, Colby T, Ordonez N, et al. Guidelines for pathologic diagnosis of malignant mesothelioma: 2012 update of the consensus statement from the International Mesothelioma Interest Group. Arch Pathol Lab Med 2013; 137:647.
  9. Rusch VW, Giroux D, Kennedy C, et al. Initial analysis of the international association for the study of lung cancer mesothelioma database. J Thorac Oncol 2012; 7:1631.
  10. AJCC (American Joint Committee on Cancer) Cancer Staging Manual, 8th edition, Amin MB, Edge SB, Greene FL et al. (Eds), Springer, Chicago 2017.
  11. Richards WG. Recent advances in mesothelioma staging. Semin Thorac Cardiovasc Surg 2009; 21:105.
  12. Heelan RT, Rusch VW, Begg CB, et al. Staging of malignant pleural mesothelioma: comparison of CT and MR imaging. AJR Am J Roentgenol 1999; 172:1039.
  13. Marom EM, Erasmus JJ, Pass HI, Patz EF Jr. The role of imaging in malignant pleural mesothelioma. Semin Oncol 2002; 29:26.
  14. Miller WT Jr, Gefter WB, Miller WT Sr. Asbestos-related chest diseases: plain radiographic findings. Semin Roentgenol 1992; 27:102.
  15. Sørensen JB, Ravn J, Loft A, et al. Preoperative staging of mesothelioma by 18F-fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography fused imaging and mediastinoscopy compared to pathological findings after extrapleural pneumonectomy. Eur J Cardiothorac Surg 2008; 34:1090.
  16. Plathow C, Staab A, Schmaehl A, et al. Computed tomography, positron emission tomography, positron emission tomography/computed tomography, and magnetic resonance imaging for staging of limited pleural mesothelioma: initial results. Invest Radiol 2008; 43:737.
  17. Wilcox BE, Subramaniam RM, Peller PJ, et al. Utility of integrated computed tomography-positron emission tomography for selection of operable malignant pleural mesothelioma. Clin Lung Cancer 2009; 10:244.
  18. Rusch VW, Gill R, Mitchell A, et al. A Multicenter Study of Volumetric Computed Tomography for Staging Malignant Pleural Mesothelioma. Ann Thorac Surg 2016; 102:1059.
  19. Rice DC, Erasmus JJ, Stevens CW, et al. Extended surgical staging for potentially resectable malignant pleural mesothelioma. Ann Thorac Surg 2005; 80:1988.
  20. Sugarbaker DJ, Jaklitsch MT, Liptay MJ. Mesothelioma and radical multimodality therapy: who benefits? Chest 1995; 107:345S.
  21. Herndon JE, Green MR, Chahinian AP, et al. Factors predictive of survival among 337 patients with mesothelioma treated between 1984 and 1994 by the Cancer and Leukemia Group B. Chest 1998; 113:723.
  22. Edwards JG, Abrams KR, Leverment JN, et al. Prognostic factors for malignant mesothelioma in 142 patients: validation of CALGB and EORTC prognostic scoring systems. Thorax 2000; 55:731.
  23. Mikulski SM, Costanzi JJ, Vogelzang NJ, et al. Phase II trial of a single weekly intravenous dose of ranpirnase in patients with unresectable malignant mesothelioma. J Clin Oncol 2002; 20:274.
  24. Vogelzang NJ, Rusthoven JJ, Symanowski J, et al. Phase III study of pemetrexed in combination with cisplatin versus cisplatin alone in patients with malignant pleural mesothelioma. J Clin Oncol 2003; 21:2636.
  25. Ahamad A, Stevens CW, Smythe WR, et al. Promising early local control of malignant pleural mesothelioma following postoperative intensity modulated radiotherapy (IMRT) to the chest. Cancer J 2003; 9:476.
  26. Krug LM, Pass HI, Rusch VW, et al. Multicenter phase II trial of neoadjuvant pemetrexed plus cisplatin followed by extrapleural pneumonectomy and radiation for malignant pleural mesothelioma. J Clin Oncol 2009; 27:3007.
  27. Yang CF, Adil SM, Anderson KL, et al. Impact of patient selection and treatment strategies on outcomes after lobectomy for biopsy-proven stage IIIA pN2 non-small cell lung cancer. Eur J Cardiothorac Surg 2016; 49:1607.
  28. Musk AW, Olsen N, Alfonso H, et al. Predicting survival in malignant mesothelioma. Eur Respir J 2011; 38:1420.
  29. Curran D, Sahmoud T, Therasse P, et al. Prognostic factors in patients with pleural mesothelioma: the European Organization for Research and Treatment of Cancer experience. J Clin Oncol 1998; 16:145.
  30. Fennell DA, Parmar A, Shamash J, et al. Statistical validation of the EORTC prognostic model for malignant pleural mesothelioma based on three consecutive phase II trials. J Clin Oncol 2005; 23:184.
  31. Bottomley A, Coens C, Efficace F, et al. Symptoms and patient-reported well-being: do they predict survival in malignant pleural mesothelioma? A prognostic factor analysis of EORTC-NCIC 08983: randomized phase III study of cisplatin with or without raltitrexed in patients with malignant pleural mesothelioma. J Clin Oncol 2007; 25:5770.
  32. Gordon GJ, Jensen RV, Hsiao LL, et al. Using gene expression ratios to predict outcome among patients with mesothelioma. J Natl Cancer Inst 2003; 95:598.
  33. Pass HI, Liu Z, Wali A, et al. Gene expression profiles predict survival and progression of pleural mesothelioma. Clin Cancer Res 2004; 10:849.
  34. Chang K, Pai LH, Pass H, et al. Monoclonal antibody K1 reacts with epithelial mesothelioma but not with lung adenocarcinoma. Am J Surg Pathol 1992; 16:259.
  35. Ordóñez NG. Value of mesothelin immunostaining in the diagnosis of mesothelioma. Mod Pathol 2003; 16:192.
  36. Hollevoet K, Reitsma JB, Creaney J, et al. Serum mesothelin for diagnosing malignant pleural mesothelioma: an individual patient data meta-analysis. J Clin Oncol 2012; 30:1541.
  37. Scherpereel A, Grigoriu B, Conti M, et al. Soluble mesothelin-related peptides in the diagnosis of malignant pleural mesothelioma. Am J Respir Crit Care Med 2006; 173:1155.
  38. Creaney J, Yeoman D, Naumoff LK, et al. Soluble mesothelin in effusions: a useful tool for the diagnosis of malignant mesothelioma. Thorax 2007; 62:569.
  39. Davies HE, Sadler RS, Bielsa S, et al. Clinical impact and reliability of pleural fluid mesothelin in undiagnosed pleural effusions. Am J Respir Crit Care Med 2009; 180:437.
  40. Pass HI, Levin SM, Harbut MR, et al. Fibulin-3 as a blood and effusion biomarker for pleural mesothelioma. N Engl J Med 2012; 367:1417.
  41. Pass HI, Lott D, Lonardo F, et al. Asbestos exposure, pleural mesothelioma, and serum osteopontin levels. N Engl J Med 2005; 353:1564.
  42. Hiraki A, Aoe K, Ueoka H. Asbestos exposure and serum osteopontin. N Engl J Med 2006; 354:304.
  43. Muñoz A, Barceló R, López-Vivanco G. Malignant mesothelioma. N Engl J Med 2006; 354:305.
  44. Grigoriu BD, Scherpereel A, Devos P, et al. Utility of osteopontin and serum mesothelin in malignant pleural mesothelioma diagnosis and prognosis assessment. Clin Cancer Res 2007; 13:2928.