• Vangelis Karamitrousis
  • Nikolaos Tsoukalas


Malignant pleural mesothelioma (MPM) is a rare cancer, linked to asbestos exposure. The median age at diagnosis is 70 years, however it can develop in younger patients. Poor prognostic factors include: pleural disease extent, high lactate dehydrogenase, poor performance status, high platelet count, non-epithelial histology, and old age. The key inactivated driver genes are CDKN2A, NF2 and BAP1. The most common symptom is dyspnea. Pleural effusion is present in most cases of MPM, revealed by a chest X-ray. Other diagnostic procedures include ultrasonography, computed tomgraphy, pleural effusion cytology, fluorodeoxyglucose-positron emission tomography and magnetic resonance imaging. There are three major histological subtypes of MPM: epithelioid, sarcomatoid and biphasic. Treatment of MPM includes surgery, radiotherapy and systemic therapy. Thoracoscopy aids in the diagnosis and management of MPM, especially in patients with large pleural effusions. Extrapleural pneumonectomy, pleurectomy and decortication, and palliative limited pleurectomy are the surgical operations used in the treatment of MPM. There is little benefit to adding radiotherapy to other treatments. The most common used systemic therapy regimens include a combination doublet of cisplatin, with an antifolate.


Mesothelioma Asbestos Trimodality therapy 



(Centi) Gray


Active symptoms control


BRCA-1 associated protein-1


Cyclin-dependent kinase inhibitor 2A/alternative reading frame


Carcinoembryonic antigen




Computer tomography


Deep venous thrombosis


Eastern Cooperative Oncology Group


Ejection fraction


Fludeoxy-glucose positron emission tomography


Forced expiratory volume in the first second




Intensity-modulated radiotherapy


Malignant pleural mesothelioma


Magnetic resonance imaging


Mitomycin, vinblastin, cisplatin


Neurofibromatosis type-2


Non-small cell lung cancer


Overall response rate


Overall survival


Pleurectomy and decortication


Predicted postoperative


Performance status




Simian virus 40


Tumor necrosis factor-α


Tumor, nodal and metastasis


Thyroid transcription factor-1




Wilm’s tumor gene product


  1. 1.
    Ismail-Khan R, Robinson LA, Williams CC Jr et al (2006) Malignant pleural mesothelioma: a comprehensive review. Cancer Control 13:255–263CrossRefGoogle Scholar
  2. 2.
    Mutsaers SE (2004) The mesothelial cell. Int J Biochem Cell Biol 36(1):9–16CrossRefGoogle Scholar
  3. 3.
    Thorgeirsson T, Isaksson HJ, Hardardottir H, Alfredsson H, Gudbjartsson T (2010) Solitary fibrous tumors of the pleura: an estimation of population incidence. Chest 137(4):1005–1006. Scholar
  4. 4.
    Porret E, Madelaine J, Galateau-Salle F et al (2007) Epidemiology, molecular biology, diagnostic and therapeutic strategy of malignant pleural mesothelioma in 2007-an update. Rev Mal Respir 24:6S157–6S164CrossRefGoogle Scholar
  5. 5.
    Robinson BWS, Musk AW, Lake RA (2005) Malignant mesothelioma. Lancet 366(9483):397–408CrossRefGoogle Scholar
  6. 6.
    Tada Y, Shimada H, Hiroshima K, Tagawa M (2013) A potential strategy for malignant mesothelioma with gene medicine. Biomed Res Int 2013:1–8CrossRefGoogle Scholar
  7. 7.
    Carbone M, Ly BH, Dodson RF et al (2012) Malignant mesothelioma: facts, myths, and hypotheses. J Cell Physiol 18:598–604. Scholar
  8. 8.
    Diseases of the pleura and mediastinum (2014) In: Niederhuber JE, Armitage JO, Doroshow JH, Kastan MB, Tepper JE (eds) Abeloff’s Clinical Oncology, 5th edn. Elsevier, PhiladelphiaGoogle Scholar
  9. 9.
    Ventii KH, Devi NS, Friedrich KL et al (2008) BRCA1-associated protein-1 is a tumor suppressor that requires deubiquitinating activity and nuclear localization. Cancer Res 68:6953–6962. Scholar
  10. 10.
    Mineo TC, Ambrogi V (2012) Malignant pleural mesothelioma: factors influencing the prognosis. Oncology (Williston Park) 26(12):1164–1175Google Scholar
  11. 11.
    Sekido Y (2013) Molecular genesis of malignant mesothelioma. Carcinogenesis 34(7):1413–1419. Epub 2013 May 14CrossRefPubMedGoogle Scholar
  12. 12.
    Steele JPC, Klabatsa A, Fennell DA et al (2005) Prognostic factors in mesothelioma. Lung Cancer 49(Suppl 1):S49–S52CrossRefGoogle Scholar
  13. 13.
    Craighead J (2011) Epidemiology of mesothelioma and historical background. Recent Results Cancer Res 189:13–25. Scholar
  14. 14.
    Champbell N, Kindler H (2011) Update on malignant pleural mesothelioma. Semin Respir Crit Care Med 32:102–110. Epub 2011 Apr 15.CrossRefGoogle Scholar
  15. 15.
    Antman KH (1981) Clinical presentation and natural history of benign and malignant mesothelioma. Semin Oncol 8:313–320PubMedGoogle Scholar
  16. 16.
    Neumann V, Günther S, Müller K, Fischer M (2001) Malignant mesothelioma – German mesotheliom register 1987–1999. Int Arch Occup Health 74:383–395CrossRefGoogle Scholar
  17. 17.
    De Pangher Manzini V, Brollo A, Bianchi C (1990) Thrombocytosis in malignant pleural mesothelioma. Tumori 76:576–578CrossRefGoogle Scholar
  18. 18.
    Neumann V, Löseke S, Nowak D, Herth FJ, Tannapfel A (2013) Malignant pleural mesothelioma: incidence, etiology, diagnosis, treatment, and occupational health. Dtsch Arztebl Int 110(18):319–326. Epub 2013 May 3CrossRefPubMedPubMedCentralGoogle Scholar
  19. 19.
    Tsujimura T, Torii I, Sato A et al (2012) Pathological and molecular biological approaches to early mesothelioma. Int J Clin Oncol 17:40–47. Epub 2012 Jan 12CrossRefPubMedGoogle Scholar
  20. 20.
    Curran D, Sahmoud T, Therasse P et al (1998) Prognostic factors in patients with pleural mesothelioma: the European Organization for research and treatment of cancer experience. J Clin Oncol 16:145–152CrossRefGoogle Scholar
  21. 21.
    Bottomley A, Coens C, Efficace F et al (2007) 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 25:5770–5776CrossRefGoogle Scholar
  22. 22.
    Travis WD, Bramilla E, Muller-Hermelink et al (2004) World Health Organization Classification of Tumors. Pathology and genetics of tumours of the lung, pleura, thymus and heart, 4th edn. IARC Press Lyon, FranceGoogle Scholar
  23. 23.
    Van Zandwijk N, Clarke C, Henderson D, Musk AW, Fong K, Nowak A, Loneragan R, McCaughan B, Boyer M, Feigen M, Currow D, Schofield P, Ivimey B, Pavlakis N, McLean J, Marshall H, Leong S, Keena V, Penman A (2013) Guidelines for the diagnosis and treatment of malignant pleural msothelioma. J Thorac Dis 5(6):E254–E307. Scholar
  24. 24.
    Ghanim B, Hoda MA, Klikovits T, Winter MP, Alimohammadi A, Grusch M, Dome B, Arns M, Schenk P, Jakopovic M, Samarzija M, Brcic L, Filipits M, Laszlo V, Klepetko W, Berger W, Hegedus B (2014) Circulating fibrinogen is a prognostic and predictive biomarker in malignant pleural mesothelioma. Br J Cancer 110(4):984–990. Epub 2014 Jan 16CrossRefPubMedPubMedCentralGoogle Scholar
  25. 25.
    Conlon KC, Rusch VW, Gillern S (1996) Laparoscopy: an important tool in the staging of malignant pleural mesothelioma. Ann Surg Oncol 3:489–494CrossRefGoogle Scholar
  26. 26.
    Colice GL, Shafazand S, Griffin JP et al (2007) Physiologic evaluation of the patient with lung cancer being considered for resectional surgery: ACCP evidenced-based clinical practice guidelines (2nd edition). Chest 132:161S–177SCrossRefGoogle Scholar
  27. 27.
    Wolf AS, Daniel J, Sugarbaker DJ (2009) Surgical techniques for multimodality treatment of malignant pleural mesothelioma: extrapleural pneumonectomy and pleurectomy/decortication. Semin Thorac Cardiovasc Surg 21:132–148. Scholar
  28. 28.
    Sugarbaker DJ (2006) Macroscopic complete resection: the goal of primary surgery in multimodality therapy for pleural mesothelioma. J Thorac Oncol 1:175–176CrossRefGoogle Scholar
  29. 29.
    Vogelzang NJ, Rusthoven JJ, Symanowski J et al (2003) Phase III study of pemetrexed in combination with cisplatin versus cisplatin alone in patients with malignant pleural mesothelioma. J Clin Oncol 21:2636–2644CrossRefGoogle Scholar
  30. 30.
    van Meerbeeck JP, Gaafar R, Manegold C et al (2005) Randomized phase III study of cisplatin with or without raltitrexed in patients with malignant pleural mesothelioma: an intergroup study of the European Organisation for Research and Treatment of Cancer Lung Cancer Group and the National Cancer Institute of Canada. J Clin Oncol 23:6881–6889CrossRefGoogle Scholar
  31. 31.
    Santoro A, O’Brien ME, Stahel RA et al (2008) Pemetrexed plus cisplatin or pemetrexed plus carboplatin for chemonaive patients with malignant pleural mesothelioma: results of the International Expanded Access Program. J Thorac Oncol 3:756–763. Scholar
  32. 32.
    Muers MF, Stephens RJ, Fisher P et al (2008) Active symptom control with or without chemotherapy in the treatment of patients with malignant pleural mesothelioma (MS01): a multicentre randomised trial. Lancet 371:1685–1694. Scholar
  33. 33.
    Sorensen JB, Frank H, Palshof T (2008) Cisplatin and vinorelbine first-line chemotherapy in non-resectable malignant pleural mesothelioma. Br J Cancer 99:44–50. Epub 2008 Jun 10CrossRefPubMedPubMedCentralGoogle Scholar
  34. 34.
    Favaretto AG, Aversa SM, Paccagnella A et al (2003) Gemcitabine combined with carboplatin in patients with malignant pleural mesothelioma: a multicentric phase II study. Cancer 97:2791–2797CrossRefGoogle Scholar
  35. 35.
    Weder W, Kestenholz P, Taverna C et al (2004) Neoadjuvant chemotherapy followed by extrapleural pneumonectomy in malignant pleural mesothelioma. J Clin Oncol 22(17):3451–3457CrossRefGoogle Scholar
  36. 36.
    Rea F, Marulli G, Bortolotti L et al (2007) Induction chemotherapy, extrapleural pneumonectomy (EPP) and adjuvant hemi-thoracic radiation in malignant pleural mesothelioma (MPM): feasibility and results. Lung Cancer 57:89–95. Epub 2007 Apr 2CrossRefGoogle Scholar
  37. 37.
    Rusch VW, Rosenzweig K, Venkatraman E et al (2001) A phase II trial of surgical resection and adjuvant high-dose hemithoracic radiation for malignant pleural mesothelioma. J Thorac Cardiovasc Surg 122:788–795CrossRefGoogle Scholar
  38. 38.
    Gupta V, Mychalczak B, Krug L et al (2005) Hemithoracic radiation therapy after pleurectomy/decortication for malignant pleural mesothelioma. Int J Radiat Oncol Biol Phys 63:1045–1052. Epub 2005 Jul 28.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Vangelis Karamitrousis
    • 1
  • Nikolaos Tsoukalas
    • 2
  1. 1.Medical Oncology DepartmentUniversity General Hospital of EvrosAlexandroupolisGreece
  2. 2.Oncology Clinic, 401 General Military HospitalAthensGreece

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