Since the initial [American Society of Clinical Oncologists (ASCO)] clinical practice guideline on the treatment of malignant pleural mesothelioma (PM) was published in 2018, considerable progress has been made which has changed not only the management but even the name of this disease. This guideline update focuses on four key areas: the role of surgery, new systemic treatments, pathologic insights, and germline testing.
New randomized data have engendered many debates about the role of surgery in the multimodality management of PM. The approval of combination immunotherapy with ipilimumab plus nivolumab for newly diagnosed patients has markedly improved overall survival (OS), especially for nonepithelioid histology, and led to new questions about the choice of doublet immunotherapy, pemetrexed-platinum–based chemotherapy, chemoimmunotherapy, and other agents for the individual patient. Pathologists have identified a premalignant mesothelioma in situ (MIS) lesion, revised the nomenclature to omit the prefix “malignant” from the disease name, and highlighted the importance of subtype classification and grading because of prognostic and therapeutic implications. The discovery that patients with mesothelioma often have germline mutations, most commonly in the tumor suppressor gene BAP1, and that this not only affects cancer risk in patients and family members but also has prognostic significance suggests that universal germline testing should be offered to patients with mesothelioma.
These and other key advances have clearly changed the management and outlook of PM and have prompted this update to the 2018 ASCO mesothelioma guideline.
Guideline Questions
This clinical practice guideline addresses four overarching clinical questions: (1) Does surgery have a role in the management of mesothelioma? (2) How do clinicians integrate immunotherapy and chemotherapy into the treatment paradigm? (3) How do new histologic criteria guide diagnostic and treatment decisions? (4) Should genetic testing for pathogenic germline mutations be routinely performed in patients with mesothelioma?
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