NF2/Merlin in hereditary neurofibromatosis 2 versus cancer: biologic mechanisms and clinical associations

Rebecca Dunbar Schroeder _, Laura S. Angelo and Razelle Kurzrock

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Oncotarget. 2014; 5:67-77. https://doi.org/10.18632/oncotarget.1557

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Rebecca Dunbar Schroeder1,2, Laura S. Angelo1, Razelle Kurzrock1

1 Department of Investigational Cancer Therapeutics (Phase I Program), The University of Texas MD Anderson Cancer Center, Houston, TX,

2 Program in Experimental Therapeutics, The University of Texas Graduate School of Biomedical Sciences, Houston, TX


Rebecca Dunbar Schroeder, email:

Keywords: Neurofibromatosis 2, schwannoma, merlin, mutation

Received: October 30, 2013 Accepted: December 17, 2013 Published: December 17, 2013


Inactivating germline mutations in the tumor suppressor gene NF2 cause the hereditary syndrome neurofibromatosis 2, which is characterized by the development of neoplasms of the nervous system, most notably bilateral vestibular schwannoma. Somatic NF2 mutations have also been reported in a variety of cancers, but interestingly these mutations do not cause the same tumors that are common in hereditary neurofibromatosis 2, even though the same gene is involved and there is overlap in the site of mutations. This review highlights cancers in which somatic NF2 mutations have been found, the cell signaling pathways involving NF2/merlin, current clinical trials treating neurofibromatosis 2 patients, and preclinical findings that promise to lead to new targeted therapies for both cancers harboring NF2 mutations and neurofibromatosis 2 patients.

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