ArticleModern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc2016
TERT promoter mutations and prognosis in solitary fibrous tumor.
Article in Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 53 papers.
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Who cites it
53 citing papers in PubMed, 111 citations in OpenAlex.
- Intracranial Solitary Fibrous Tumors With Glandular and Papillary Structures: A Case Report.Clinical case reports · 2026Article
- Systemic Therapy for Solitary Fibrous Tumor.Current treatment options in oncology · 2026Review
- Solitary fibrous tumor: an updated review.Journal of pathology and translational medicine · 2026Article
- Rapid progression of recurrent intracranial solitary fibrous tumor following a previously reported TERT-wild-type primary tumor: a case report.Frontiers in oncology · 2026Article
- Spectrum and Clinical Impact of Secondary Genetic Alterations in Translocation-Associated Sarcomas.JCO precision oncology · 2025Article
- Case Report: Successful Treatment of Solitary Fibrous Tumor with Selective Internal Radiation Therapy (SIRT).Diseases (Basel, Switzerland) · 2024Article
- NAB2::STAT6 fusions and genome-wide DNA methylation profiling: Predictors of patient outcomes in meningeal solitary fibrous tumors.Brain pathology (Zurich, Switzerland) · 2024Article
- Solitary fibrous tumor within the mesorectum: literature review based on a case report of resection by transanal minimally invasive surgery (TAMIS).International journal of colorectal disease · 2024Review
- Prevalence ofCurrent issues in molecular biology · 2024Article
- Risk factors for recurrent disease after resection of solitary fibrous tumor: a systematic review.Frontiers in surgery · 2024Review
- Review
- Extra-meningeal solitary fibrous tumor: an evolving entity with chameleonic morphological diversity, a hallmark molecular alteration and unresolved issues in risk stratification assessment.Histology and histopathology · 2023Review
- Surgery for Solitary Fibrous Tumors of the Pleura: A Review of the Available Evidence.Cancers · 2023Review
- A review of solitary fibrous tumours of the orbit and ocular adnexa.Eye (London, England) · 2023Review
- Freiburg Neuropathology Case Conference : A 43-year-old Patient Presenting with a Right-sided, Slowly Progressing, Painless Exophthalmos and Ptosis.Clinical neuroradiology · 2023Article
- Prognostic analysis of extrameningeal solitary fibrous tumor using the modified Demicco model: a clinicopathologic study of 111 Chinese cases.Frontiers in oncology · 2023Article
- Association of human telomerase reverse transcriptase promoter mutation with unfavorable prognosis in glioma: A systematic review and meta-analysis.Journal of research in medical sciences : the official journal of Isfahan University of Medical Sciences · 2023Review
- Solitary fibrous tumor with IGF-II-induced non-islet cell tumor hypoglycemia: a case report and molecular characterization by next-generation sequencing.Frontiers in oncology · 2023Article
- Histopathological features of giant mediastinal tumors-a literature review.Mediastinum (Hong Kong, China) · 2023Review
- Evaluation of Alternative Risk Stratification Systems in a Large Series of Solitary Fibrous Tumors with Molecular Findings and Ki-67 Index Data: Do They Improve Risk Assessment?International journal of molecular sciences · 2022Article
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Authors and funding
7 authors at 3 institutions in 1 country.
Funding
Abstract
Solitary fibrous tumor is a mesenchymal neoplasm exhibiting a broad spectrum of biological behavior and harboring the NAB2-STAT6 fusion. Clinicopathologic parameters are currently used in risk-prediction models for solitary fibrous tumor, but the molecular determinants of malignancy in solitary fibrous tumors remain unknown. We proposed that the activation of telomere maintenance pathways confers a perpetual malignant phenotype to these tumors. Therefore, we investigated telomerase reverse transcriptase (TERT) reactivation induced by promoter mutations as a potential molecular mechanism for aggressive clinical behavior in solitary fibrous tumor. The retrospective study included tumor samples from 94 patients with solitary fibrous tumor (31 thoracic and 63 extra-thoracic). Follow-up information was available for 68 patients (median, 46 months). TERT promoter mutation analysis was performed by PCR and Sanger sequencing, and TERT mRNA expression was assessed by real-time quantitative reverse transcription PCR. Patients were stratified into clinicopathologic subgroups (high-risk (n=20), moderate-risk (n=28), and low-risk (n=46)) according to the risk-stratification model proposed by Demicco et al. TERT promoter mutations were identified in 26 of 94 (28%) solitary fibrous tumors: -124C>T in 23 tumors (88%), -124C>A in 1 tumor (4%), and -146C>T in 2 tumors (8%). Real-time quantitative reverse transcription PCR revealed that TERT mRNA expression was higher in all solitary fibrous tumors with the mutant TERT promoter than those with the wild-type TERT promoter. TERT promoter mutations were strongly associated with high-risk clinicopathologic characteristics and outcome. An adverse event (relapse, death) occurred in 16 of 68 (24%) patients, 12 with solitary fibrous tumors with TERT promoter mutations and 4 with the wild-type TERT promoter. TERT promoter mutations were strongly associated with older age (P=0.006), larger tumor size (P=0.000002), higher risk classifications (P=2.9 × 10
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.