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Telomere alterations in neurofibromatosis type 1-associated solid tumors.
Rodriguez, Fausto J; Graham, Mindy K; Brosnan-Cashman, Jacqueline A; Barber, John R; Davis, Christine; Vizcaino, M Adelita; Palsgrove, Doreen N; Giannini, Caterina; Pekmezci, Melike; Dahiya, Sonika; Gokden, Murat; Noë, Michael; Wood, Laura D; Pratilas, Christine A; Morris, Carol D; Belzberg, Allan; Blakeley, Jaishri; Heaphy, Christopher M.
  • Rodriguez FJ; Departments of Pathology, Johns Hopkins University School of Medicine, Sheikh Zayed Tower, Room M2101, 1800 Orleans Street, Baltimore, MD, 21231, USA. frodrig4@jhmi.edu.
  • Graham MK; Departments of Ophthalmology, Johns Hopkins University School of Medicine, Sheikh Zayed Tower, Room M2101, 1800 Orleans Street, Baltimore, MD, 21231, USA. frodrig4@jhmi.edu.
  • Brosnan-Cashman JA; Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins University School of Medicine, Sheikh Zayed Tower, Room M2101, 1800 Orleans Street, Baltimore, MD, 21231, USA. frodrig4@jhmi.edu.
  • Barber JR; Departments of Pathology, Johns Hopkins University School of Medicine, Sheikh Zayed Tower, Room M2101, 1800 Orleans Street, Baltimore, MD, 21231, USA.
  • Davis C; Departments of Pathology, Johns Hopkins University School of Medicine, Sheikh Zayed Tower, Room M2101, 1800 Orleans Street, Baltimore, MD, 21231, USA.
  • Vizcaino MA; Department of Epidemiology, Johns Hopkins Bloomberg School of Public Health, Baltimore, MD, USA.
  • Palsgrove DN; Departments of Pathology, Johns Hopkins University School of Medicine, Sheikh Zayed Tower, Room M2101, 1800 Orleans Street, Baltimore, MD, 21231, USA.
  • Giannini C; Departments of Pathology, Johns Hopkins University School of Medicine, Sheikh Zayed Tower, Room M2101, 1800 Orleans Street, Baltimore, MD, 21231, USA.
  • Pekmezci M; Departments of Pathology, Johns Hopkins University School of Medicine, Sheikh Zayed Tower, Room M2101, 1800 Orleans Street, Baltimore, MD, 21231, USA.
  • Dahiya S; Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins University School of Medicine, Sheikh Zayed Tower, Room M2101, 1800 Orleans Street, Baltimore, MD, 21231, USA.
  • Gokden M; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
  • Noë M; Department of Pathology, University of California San Francisco, San Francisco, CA, USA.
  • Wood LD; Department of Pathology and Immunology, Washington University, St. Louis, MO, USA.
  • Pratilas CA; University of Arkansas, Little Rock, AR, USA.
  • Morris CD; Departments of Pathology, Johns Hopkins University School of Medicine, Sheikh Zayed Tower, Room M2101, 1800 Orleans Street, Baltimore, MD, 21231, USA.
  • Belzberg A; Departments of Pathology, Johns Hopkins University School of Medicine, Sheikh Zayed Tower, Room M2101, 1800 Orleans Street, Baltimore, MD, 21231, USA.
  • Blakeley J; Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins University School of Medicine, Sheikh Zayed Tower, Room M2101, 1800 Orleans Street, Baltimore, MD, 21231, USA.
  • Heaphy CM; Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins University School of Medicine, Sheikh Zayed Tower, Room M2101, 1800 Orleans Street, Baltimore, MD, 21231, USA.
Acta Neuropathol Commun ; 7(1): 139, 2019 08 28.
Article en En | MEDLINE | ID: mdl-31462295
ABSTRACT
The presence of Alternative lengthening of telomeres (ALT) and/or ATRX loss, as well as the role of other telomere abnormalities, have not been formally studied across the spectrum of NF1-associated solid tumors. Utilizing a telomere-specific FISH assay, we classified tumors as either ALT-positive or having long (without ALT), short, or normal telomere lengths. A total of 426 tumors from 256 NF1 patients were evaluated, as well as 99 MPNST tumor samples that were sporadic or of unknown NF1 status. In the NF1-glioma dataset, ALT was present in the majority of high-grade gliomas 14 (of 23; 60%) in contrast to only 9 (of 47; 19%) low-grade gliomas (p = 0.0009). In the subset of ALT-negative glioma cases, telomere lengths were estimated and we observed 17 (57%) cases with normal, 12 (40%) cases with abnormally long, and only 1 (3%) case with short telomeres. In the NF1-associated malignant nerve sheath tumor (NF1-MPNST) set (n = 75), ALT was present in 9 (12%). In the subset of ALT-negative NF1-MPNST cases, telomeres were short in 9 (38%), normal in 14 (58%) and long in 1 (3%). In the glioma set, overall survival was significantly decreased for patients with ALT-positive tumors (p < 0.0001). In the NF1-MPNST group, overall survival was superior for patients with tumors with short telomeres (p = 0.003). ALT occurs in a subset of NF1-associated solid tumors and is usually restricted to malignant subsets. In contrast, alterations in telomere lengths are more prevalent than ALT.
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Texto completo: 1 Banco de datos: MEDLINE Asunto principal: Neoplasias Encefálicas / Telómero / Neurofibromatosis 1 / Homeostasis del Telómero Tipo de estudio: Risk_factors_studies Límite: Adult / Female / Humans / Male Idioma: En Año: 2019 Tipo del documento: Article
Texto completo
Imprimir
XML
PubMed Links
Search on Google

Texto completo: 1 Banco de datos: MEDLINE Asunto principal: Neoplasias Encefálicas / Telómero / Neurofibromatosis 1 / Homeostasis del Telómero Tipo de estudio: Risk_factors_studies Límite: Adult / Female / Humans / Male Idioma: En Año: 2019 Tipo del documento: Article