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Telomere alterations in neurofibromatosis type 1-associated solid tumors.
- Source :
- Acta Neuropathologica Communications; 8/28/2019, Vol. 7 Issue 1, pN.PAG-N.PAG, 1p
- Publication Year :
- 2019
-
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. [ABSTRACT FROM AUTHOR]
- Subjects :
- TELOMERES
TUMORS
GLIOMAS
NERVES
HUMAN abnormalities
INTERLEUKIN-9
Subjects
Details
- Language :
- English
- ISSN :
- 20515960
- Volume :
- 7
- Issue :
- 1
- Database :
- Complementary Index
- Journal :
- Acta Neuropathologica Communications
- Publication Type :
- Academic Journal
- Accession number :
- 138314598
- Full Text :
- https://doi.org/10.1186/s40478-019-0792-5