Risk assessment in solitary fibrous tumors: validation and refinement of a risk stratification model View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2017-10

AUTHORS

Elizabeth G Demicco, Michael J Wagner, Robert G Maki, Vishal Gupta, Ilya Iofin, Alexander J Lazar, Wei-Lien Wang

ABSTRACT

Solitary fibrous tumors are an uncommon sarcoma type characterized by NAB2-STAT6 gene fusion. While solitary fibrous tumors metastasize in 5-25% of cases, it has historically been challenging to determine which specific tumor and patient characteristics predict aggressive behavior. We previously reported on a novel risk stratification scheme for solitary fibrous tumors incorporating patient age, tumor size, and mitotic activity to predict risk of metastasis. Herein we validate this risk stratification scheme in an independent, lower-risk population of 79 patients with primary non-meningeal solitary fibrous tumors, and propose incorporating tumor necrosis as a fourth variable to further improve the risk score. Fifty-seven percent of cases were considered low risk, 29% intermediate risk, and 14% high risk for metastasis. Of 50 patients with sufficient clinical follow-up data, no metastases developed in the low-risk patients (n=23), while there was a 7% 10-year metastatic risk in the intermediate risk group (n=17), and a 49% 5-year metastatic risk for the high-risk patients (n=10). When tumor necrosis was added as a fourth variable to the model, predictive power was enhanced. Under the revised stratification, the proportion of tumors identified as low risk increased to 66%, with no metastasis at 10 years, intermediate risk cases comprised 24% with 10% risk of metastasis at 10 years, and high risk comprised 10% of cases with 73% risk of metastasis at 5 years. In Kaplan-Meier analysis, this fourth-variable stratification provided significant discrimination between the risk groups (P=0.0005). These findings confirmed the clinical utility of our previously published risk stratification model and support the inclusion of necrosis as a fourth variable in the model. More... »

PAGES

1433-1442

References to SciGraph publications

  • 2013-02. Identification of recurrent NAB2-STAT6 gene fusions in solitary fibrous tumor by integrative sequencing in NATURE GENETICS
  • 2016-12. TERT promoter mutations and prognosis in solitary fibrous tumor in MODERN PATHOLOGY
  • 2012-09. Solitary fibrous tumor: a clinicopathological study of 110 cases and proposed risk assessment model in MODERN PATHOLOGY
  • 2013-12. Distribution of TERT promoter mutations in pediatric and adult tumors of the nervous system in ACTA NEUROPATHOLOGICA
  • 2014-12. TERT promoter hotspot mutations are recurrent in myxoid liposarcomas but rare in other soft tissue sarcoma entities in JOURNAL OF EXPERIMENTAL & CLINICAL CANCER RESEARCH
  • 2015-10. NAB2–STAT6 fusion types account for clinicopathological variations in solitary fibrous tumors in MODERN PATHOLOGY
  • 2015-12. Comprehensive screening of alternative lengthening of telomeres phenotype and loss of ATRX expression in sarcomas in MODERN PATHOLOGY
  • 2013-02. Whole-exome sequencing identifies a recurrent NAB2-STAT6 fusion in solitary fibrous tumors in NATURE GENETICS
  • 2013-12. Predicting Behavior of Solitary Fibrous Tumor: Are We Getting Closer to More Accurate Risk Assessment? in ANNALS OF SURGICAL ONCOLOGY
  • 2001-01. Accuracy of Biopsy Techniques for Limb and Limb Girdle Soft Tissue Tumors in ANNALS OF SURGICAL ONCOLOGY
  • 2013-12. Prognosis of Solitary Fibrous Tumors: A Multicenter Study in ANNALS OF SURGICAL ONCOLOGY
  • 2013-12. Extrathoracic Location and “Borderline” Histology are Associated with Recurrence of Solitary Fibrous Tumors After Surgical Resection in ANNALS OF SURGICAL ONCOLOGY
  • Identifiers

    URI

    http://scigraph.springernature.com/pub.10.1038/modpathol.2017.54

    DOI

    http://dx.doi.org/10.1038/modpathol.2017.54

    DIMENSIONS

    https://app.dimensions.ai/details/publication/pub.1090853041

    PUBMED

    https://www.ncbi.nlm.nih.gov/pubmed/28731041


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