Manegold, C.

Stahel, R. A.

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Minimum Clinical Recommendations for diagnosis, treatmentand follow-up of malignant pleural mesotheliomaIncidence. Malignant pleural mesothelioma (MPM) is a rare tumor.The incidence is 1.25/100 000 in UK and 1.1/100 000 inGermany. Within the next 20 years the incidence is esti-mated to double. Asbestos is a well established etiologicalfactor for MPM, with occupational exposure in 70–80% ofthose affected.Diagnosis. Patients typically present with chest pain or increasingshortness of breath. The diagnosis is usually suggested byimaging studies (unilateral pleural mass; pleural effusion).Occupational history must be obtained.. Cytological examination of the effusion or pleural biopsycan be diagnostic, but often shows equivocal results. Video-assisted thoracoscopy or open pleural biopsy may be necess-ary to provide sufficient material. There are three main his-tological types (epithelial, sarcomatous, and mixed) withabout 60% being epithelial.Staging and risk assessment. Staging includes complete history, physical examination,and chest X-ray. If local therapy is being considered a CTscan of the chest should be obtained. Accurate initial sta-ging is important as this will provide both prognostic infor-mation and suggest the most appropriate therapeuticoptions. The new international staging system for MPMemphasizes the extent of disease in a traditional TNM sys-tem and stratifies patients into similar prognostic categories(see Table 1).. The Cancer and Leukemia Group B (CALGB) and theEORTC prognostic scores may be used. They include per-formance status, age, histological type, weight loss, andwhite blood count.. MPM rarely metastasize to distant sites but most patientspresent with locally advanced disease.TreatmentSurgery. A variety of surgical procedures have been tried with vari-able success.. Extra-pleural pneumonectomy with excision of the dia-phragm and the pericardium en bloc has the potential ofcomplete removal of the tumor. This approach is generallycombined with chemotherapy and/or radiotherapy andshould only be attempted by expert surgeons and chestoncology teams [III, A].. Palliative local procedures include parietal pleurectomy,decortication or pleurodesis.Radiotherapy. The use of conventional radiotherapy has been limitedbecause it is impossible to avoid high-dose irradiation of theunderlying lung. Conventional dose can be delivered in apalliative attempt. Modern radiotherapy techniques can deli-ver high dose radiotherapy with curative intent after extra-pleural pneumonectomy. Prophylactic radiotherapy has beenshown to reduce the incidence of port metastases [II, A].Chemotherapy. Platinum analogues, doxorubicin and several antimetabolites(methotrexate, etatrexate, raltitrexate, pemetrexed) haveshown modest single agent activity [III, B].. Better symptom improvement may be obtained with gemci-tabine in combination with cisplatin [III, A].. The combination of pemetrexed and cisplatin improved sur-vival and quality of life in comparison to cisplatin alone ina randomized trial [II, A].. Available date indicate a similar management for extra-pleural malignant mesothelioma [IV, A].Follow-up. Follow-up consists of clinical evaluation with particularattention to symptoms and chest-CT as needed.NoteLevels of Evidence [I–V] and Grades of Recommendation[A–D] as used by the American Society of Clinical Oncologyare given in square brackets. Statements without grading wereconsidered justified standard clinical practice by the expertauthors and the ESMO faculty.Annals of Oncology 16 (Supplement 1): i32–i33, 2005doi:10.1093/annonc/mdi835q 2005 European Society for Medical OncologyLiterature1. Peto J, Decarli A, La Vecchia C et al. The European mesothelioma epi-demic. Br J Cancer 1999; 86: 1970–1971.2. Pelucchi C, Malvezzi M, La Vecchia C et al. The mesothelioma epi-demic in Western Europe: an update. Br J Cancer 2004; 90:1022–1024.3. Sugarbaker DJ, Flores RM, Jaklitsch MT et al. Resection margins,extrapleural nodal status, and cell type determine postoperative long-term survival in trimodality therapy of malignant pleural mesothe-lioma: results in 183 patients. J Thorac Cardiovasc Surg 1999; 17:54–63.4. Weder W, Kestenholz P, Taverna C et al. Neoadjuvant chemotherapyfollowed by extrapleural pneumonectomy in malignant pleuralmesothelioma. J Clin Oncol 2004; 22: 3451–3457.5. Tomek S, Emri S, Krejcy E, Manegold C. Chemotherapy for malignantpleural mesothelioma: Past results and recent developments. Br J Can-cer 2003; 88: 167–174.6. Senan S. Indications and limitations of radiotherapy in malignantpleural mesothelioma. Current Opinion in Oncology 2003; 15:144–147.7. Vogelzang NJ, Rusthoven JJ, Symanowski J et al. Phase III study ofpemetrexed in combination with cisplatin versus cisplatin alone inpatients with malignant pleural mesothelioma. J Clin Oncol 2003; 21:2636–2644.8. Waller DA. The role of surgery in diagnosis and treatment of malig-nant pleural mesothelioma. Current Opinion in Oncology 2003; 15:139–143.Coordinating authors for the ESMO Guidelines Task Force: C. Manegold1& R. A. Stahel21Invited author, Thoraxklinik Amalienstr. 5, D-69126 Heidelberg,Germany; 2Assigned task force member, Div. of Oncology, UniversityHospital, Ra¨mistr. 100, CH-8091 Zu¨rich, SwitzerlandApproved by the ESMO Guidelines Task Force: December 2004.Correspondence to:ESMO Guidelines Task ForceESMO Head OfficeVia La Santa 7CH-6962 LuganoSwitzerlandTable 1.Stage TNM CommentsIa T1a N0 M0 Primary tumor limited to ipsilateral parietal pleuraIb T1b N0 M0 As stage Ia plus focal involvement of visceral pleuraII T2 N0 M0 As stage Ia or Ib plus confluent involvement of diaphragm or visceral pleura or involvement of the lungIII any T3 M0, any N1 M0,any N2 M0Locally advanced, potentially resectable tumor; ipsilateral, bronchopulmonary or hilarlymph node involvement; subcarinal or ipsilateral mediastinal lymph node involvementIV any T4, any N3, any M1 Locally advanced technically unresectable tumor; contralateral mediastinal, internal mammary,and ipsilateral or contralateral supraclavicular lymph node involvement; distant metastasesi33

Minimum Clinical Recommendations for diagnosis, treatment and follow-up of malignant pleural mesothelioma

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