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Updated: Jun 4, 2026
Translational Orthotopic Models of Glioblastoma Multiforme
Published on: February 17, 2023
Glioblastoma multiforme in the Muir-Torre syndrome
Zev A Binder1, Michael W Johnson, Avadhut Joshi
1Department of Neurosurgery, Johns Hopkins School of Medicine, Baltimore, MD 21287, USA.
Abstract:
Muir-Torre syndrome (MTS) is an autosomal dominant subtype of nonpolyposis colorectal carcinoma (HNPCC) characterized by the development of sebaceous gland tumors and visceral malignancies. The most common subtype of MTS is characterized by germline mutations in mismatch repair (MMR) genes leading to microsatellite instability (MSI). Central nervous system tumors have only rarely been associated with MTS. In this report, we describe the development of a glioblastoma multiforme (GBM) in a patient with MTS. Immunohistochemical analysis of the patient's colon carcinoma and his GBM both revealed loss of the mismatch repair proteins mutS homolog 2 (MSH2) and mutS homolog 6 (MSH6).
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