New Strategies in Pleural Mesothelioma: BAP1 and NF2 as Novel Targets for Therapeutic Development and Risk Assessment

• Published in: Clinical cancer research Vol. 18; no. 17; pp. 4485 - 4490
• Main Authors: LADANYI, Marc, ZAUDERER, Marjorie G, KRUG, Lee M, ITO, Tatsuo, MCMILLAN, Robert, BOTT, Matthew, GIANCOTTI, Filippo
• Format: Journal Article
• Language: English
• Published: Philadelphia, PA American Association for Cancer Research 01.09.2012
• Subjects: Antineoplastic agents; Biological and medical sciences; Genetic Predisposition to Disease; Germ-Line Mutation; Humans; Medical sciences; Mesothelioma - genetics; Mesothelioma - metabolism; Mesothelioma - therapy; Molecular Targeted Therapy; Neurofibromatosis 2 - genetics; Neurofibromatosis 2 - metabolism; Pharmacology. Drug treatments; Risk Assessment; Tumor Suppressor Proteins - genetics; Tumor Suppressor Proteins - metabolism; Ubiquitin Thiolesterase - genetics; Ubiquitin Thiolesterase - metabolism; Evaluation; Treatment; Targeting; Targeted therapy; Risk factor; Development; Tumor; Mesothelioma; Pleura; Research and development
• ISSN: 1078-0432; 1557-3265
• DOI: 10.1158/1078-0432.ccr-11-2375

Malignant pleural mesothelioma (MPM) is a highly lethal cancer with limited therapeutic options. Recent work has focused on the frequent somatic inactivation of two tumor suppressor genes in MPM-NF2 (Neurofibromatosis type 2) and the recently identified BAP1 (BRCA associated protein 1). In addition, germline mutations in BAP1 have been identified that define a new familial cancer syndrome, which includes MPM, ocular melanoma, and other cancers. These recent advances may allow screening of high-risk individuals and the development of new therapies that target key pathways in MPM.