Diagnosis and Staging: Genomic studies are not recommended in routine clinical practice but may be appropriate in selected patients with malignant pleural mesothelioma (MPM), especially those with young age and family history of cancer, who may carry germline mutations in cancer susceptibility genes.
Chest computed tomography (CT) scan with intravenous contrast should be performed as the initial evaluation in patients with suspected MPM.
The diagnosis of MPM should always be based on the results obtained from an adequate biopsy in the context of appropriate clinical, radiologic, and surgical findings.
Treatment: For MPM patients with symptomatic pleural effusions who are candidates for palliative chemotherapy, complete drainage of the pleural space with subsequent pleurodesis is recommended [ in routine clinical practice. ]
In selected patients with early-stage MPM (confined to pleural envelope, no N2 lymph node involvement) and epithelioid histology, surgical interventions for maximal cytoreduction with macroscopic complete resection of all tumor include extrapleural pneumonectomy (EPP) and pleurectomy/decortication (P/D). These procedures should be performed in highly specialized centers by experienced thoracic surgeons.
Prophylactic radiation therapy (RT) of chest wall procedure tracts should not be routinely offered [ in clinical practice. ]
The role of RT as part of multimodality treatment is controversial and due to intensive toxicity RT should be performed in highly specialized centers by experienced radiation oncologists.
Patients with MPM who are not candidates for surgery should receive first-line treatment with platinum-based chemotherapy. In patients who cannot tolerate cisplatin, carboplatin might be used in combination with pemetrexed. Maintenance therapy with pemetrexed does not improve overall survival. The standard of care consists of 4–6 cycles of cisplatin plus pemetrexed.
For second-line therapy, retreatment with pemetrexed-based chemotherapy can be considered in MPM patients who achieved durable disease control (> 6 months) with first-line pemetrexed-based chemotherapy. Second-line pemetrexed is recommended in patients previously treated with first-line chemotherapy regimens that did not include pemetrexed. Second-line chemotherapy with gemcitabine or vinorelbine can be offered. Participation in clinical trials should be encouraged due to the limited efficacy of second-line chemotherapy.
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