Histological and molecular features of solitary fibrous tumor of the extremities: clinical correlation View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2019-08-28

AUTHORS

Giuseppe Bianchi, Andrea Sambri, Elena Pedrini, Laura Pazzaglia, Luca Sangiorgi, Poosit Ruengwanichayakun, Davide Donati, Maria Serena Benassi, Alberto Righi

ABSTRACT

Solitary fibrous tumor is a rare mesenchymal neoplasm that exhibits a broad spectrum of biological behaviors. Few studies relative to clinical-pathologic features and predictive factors have been reported, all involving a mixed population of tumors occurring at different anatomic sites. In this study, we described a cohort of 41 patients with solitary fibrous tumor of the extremities and evaluated the prognostic role of clinical and histological features, presence of C228T and C250T mutations at the TERT promoter region, and NAB2–STAT6 fusion variants. Patients were stratified according to the latest risk stratification model proposed by Demicco. The two patients with metastasis at presentation were in the high-risk group; the one with metastasis after surgery was classified in the intermediate-risk group. TERT promoter mutations were detected in 9 out of 38 DNA available. All patients with metastasis were characterized by a TERT promoter mutation. TERT promoter mutation was associated with mitoses > 4 per high-power field (p = 0.001), necrosis (p = 0.049), and size > 10 cm (p = 0.031). NAB2–STAT6 fusion variants were detected in 27 out of 41 cases without any prognostic value. In conclusion, we confirmed that the patients with solitary fibrous tumor of the limbs have a better prognosis than other solitary fibrous tumors, with a very low percentage of metastatic events. Besides, our data support an association between TERT promoter mutations and histologically malignant features, suggesting a possible molecular role in stratifying patients into intermediate- to high-risk tumor. More... »

PAGES

445-454

References to SciGraph publications

  • 2013-01-13. Identification of recurrent NAB2-STAT6 gene fusions in solitary fibrous tumor by integrative sequencing in NATURE GENETICS
  • 2010-10-16. Solitary fibrous tumor: an update on the spectrum of extrapleural manifestations in SKELETAL RADIOLOGY
  • 2012-05-11. Solitary fibrous tumor: a clinicopathological study of 110 cases and proposed risk assessment model in MODERN PATHOLOGY
  • 2014-04-11. TERT promoter hotspot mutations are recurrent in myxoid liposarcomas but rare in other soft tissue sarcoma entities in JOURNAL OF EXPERIMENTAL & CLINICAL CANCER RESEARCH
  • 2017-07-21. Risk assessment in solitary fibrous tumors: validation and refinement of a risk stratification model in MODERN PATHOLOGY
  • 2016-08-26. TERT promoter mutations and prognosis in solitary fibrous tumor in MODERN PATHOLOGY
  • 2015-07-31. NAB2–STAT6 fusion types account for clinicopathological variations in solitary fibrous tumors in MODERN PATHOLOGY
  • 2014-11-29. Solitary fibrous tumor – clinicopathologic, immunohistochemical and molecular analysis of 28 cases in DIAGNOSTIC PATHOLOGY
  • 2013-01-13. Whole-exome sequencing identifies a recurrent NAB2-STAT6 fusion in solitary fibrous tumors in NATURE GENETICS
  • 2017-10-16. Size and Location are the Most Important Risk Factors for Malignant Behavior in Resected Solitary Fibrous Tumors in ANNALS OF SURGICAL ONCOLOGY
  • 2019-02-02. Prognostic significance of NAB2–STAT6 fusion variants and TERT promotor mutations in solitary fibrous tumors/hemangiopericytomas of the CNS: not (yet) clear in ACTA NEUROPATHOLOGICA
  • 2013-09-18. 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.10.1007/s00428-019-02650-5

    DOI

    http://dx.doi.org/10.1007/s00428-019-02650-5

    DIMENSIONS

    https://app.dimensions.ai/details/publication/pub.1120656632

    PUBMED

    https://www.ncbi.nlm.nih.gov/pubmed/31463729


    Indexing Status Check whether this publication has been indexed by Scopus and Web Of Science using the SN Indexing Status Tool
    Incoming Citations Browse incoming citations for this publication using opencitations.net

    JSON-LD is the canonical representation for SciGraph data.

    TIP: You can open this SciGraph record using an external JSON-LD service: JSON-LD Playground Google SDTT

    [
      {
        "@context": "https://springernature.github.io/scigraph/jsonld/sgcontext.json", 
        "about": [
          {
            "id": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/11", 
            "inDefinedTermSet": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/", 
            "name": "Medical and Health Sciences", 
            "type": "DefinedTerm"
          }, 
          {
            "id": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/1112", 
            "inDefinedTermSet": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/", 
            "name": "Oncology and Carcinogenesis", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Adolescent", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Adult", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Aged", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Aged, 80 and over", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Extremities", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Female", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Humans", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Male", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Middle Aged", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Mutation", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Solitary Fibrous Tumors", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Telomerase", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Young Adult", 
            "type": "DefinedTerm"
          }
        ], 
        "author": [
          {
            "affiliation": {
              "alternateName": "Department of Orthopedic Oncology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy", 
              "id": "http://www.grid.ac/institutes/grid.419038.7", 
              "name": [
                "Department of Orthopedic Oncology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy"
              ], 
              "type": "Organization"
            }, 
            "familyName": "Bianchi", 
            "givenName": "Giuseppe", 
            "id": "sg:person.016345573425.18", 
            "sameAs": [
              "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.016345573425.18"
            ], 
            "type": "Person"
          }, 
          {
            "affiliation": {
              "alternateName": "Department of Orthopedic Oncology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy", 
              "id": "http://www.grid.ac/institutes/grid.419038.7", 
              "name": [
                "Department of Orthopedic Oncology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy"
              ], 
              "type": "Organization"
            }, 
            "familyName": "Sambri", 
            "givenName": "Andrea", 
            "id": "sg:person.01021210240.50", 
            "sameAs": [
              "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01021210240.50"
            ], 
            "type": "Person"
          }, 
          {
            "affiliation": {
              "alternateName": "Department of Medical Genetics and Rare Orthopedic Diseases, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy", 
              "id": "http://www.grid.ac/institutes/grid.419038.7", 
              "name": [
                "Department of Medical Genetics and Rare Orthopedic Diseases, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy"
              ], 
              "type": "Organization"
            }, 
            "familyName": "Pedrini", 
            "givenName": "Elena", 
            "id": "sg:person.01031543327.58", 
            "sameAs": [
              "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01031543327.58"
            ], 
            "type": "Person"
          }, 
          {
            "affiliation": {
              "alternateName": "Experimental Oncology Laboratory, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy", 
              "id": "http://www.grid.ac/institutes/grid.419038.7", 
              "name": [
                "Experimental Oncology Laboratory, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy"
              ], 
              "type": "Organization"
            }, 
            "familyName": "Pazzaglia", 
            "givenName": "Laura", 
            "id": "sg:person.0746240275.84", 
            "sameAs": [
              "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0746240275.84"
            ], 
            "type": "Person"
          }, 
          {
            "affiliation": {
              "alternateName": "Department of Medical Genetics and Rare Orthopedic Diseases and CLIBI Laboratory, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy", 
              "id": "http://www.grid.ac/institutes/grid.419038.7", 
              "name": [
                "Department of Medical Genetics and Rare Orthopedic Diseases and CLIBI Laboratory, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy"
              ], 
              "type": "Organization"
            }, 
            "familyName": "Sangiorgi", 
            "givenName": "Luca", 
            "id": "sg:person.01102031714.37", 
            "sameAs": [
              "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01102031714.37"
            ], 
            "type": "Person"
          }, 
          {
            "affiliation": {
              "alternateName": "Department of Pathology, Faculty of Medicine, Naresuan University, Phitsanulok, Thailand", 
              "id": "http://www.grid.ac/institutes/grid.412029.c", 
              "name": [
                "Department of Pathology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy", 
                "Department of Pathology, Faculty of Medicine, Naresuan University, Phitsanulok, Thailand"
              ], 
              "type": "Organization"
            }, 
            "familyName": "Ruengwanichayakun", 
            "givenName": "Poosit", 
            "id": "sg:person.010507452341.44", 
            "sameAs": [
              "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.010507452341.44"
            ], 
            "type": "Person"
          }, 
          {
            "affiliation": {
              "alternateName": "Department of Orthopedic Oncology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy", 
              "id": "http://www.grid.ac/institutes/grid.419038.7", 
              "name": [
                "Department of Orthopedic Oncology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy"
              ], 
              "type": "Organization"
            }, 
            "familyName": "Donati", 
            "givenName": "Davide", 
            "id": "sg:person.01202643430.41", 
            "sameAs": [
              "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01202643430.41"
            ], 
            "type": "Person"
          }, 
          {
            "affiliation": {
              "alternateName": "Experimental Oncology Laboratory, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy", 
              "id": "http://www.grid.ac/institutes/grid.419038.7", 
              "name": [
                "Experimental Oncology Laboratory, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy"
              ], 
              "type": "Organization"
            }, 
            "familyName": "Benassi", 
            "givenName": "Maria Serena", 
            "id": "sg:person.01215431251.01", 
            "sameAs": [
              "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01215431251.01"
            ], 
            "type": "Person"
          }, 
          {
            "affiliation": {
              "alternateName": "Department of Pathology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy", 
              "id": "http://www.grid.ac/institutes/grid.419038.7", 
              "name": [
                "Department of Pathology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy"
              ], 
              "type": "Organization"
            }, 
            "familyName": "Righi", 
            "givenName": "Alberto", 
            "id": "sg:person.01115347567.94", 
            "sameAs": [
              "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01115347567.94"
            ], 
            "type": "Person"
          }
        ], 
        "citation": [
          {
            "id": "sg:pub.10.1038/modpathol.2016.126", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1003285631", 
              "https://doi.org/10.1038/modpathol.2016.126"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1007/s00256-010-1032-z", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1033624330", 
              "https://doi.org/10.1007/s00256-010-1032-z"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1186/s13000-014-0224-6", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1018093528", 
              "https://doi.org/10.1186/s13000-014-0224-6"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/ng.2509", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1049090988", 
              "https://doi.org/10.1038/ng.2509"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1186/1756-9966-33-33", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1015633596", 
              "https://doi.org/10.1186/1756-9966-33-33"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/modpathol.2015.90", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1035569641", 
              "https://doi.org/10.1038/modpathol.2015.90"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/ng.2522", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1052144262", 
              "https://doi.org/10.1038/ng.2522"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1245/s10434-013-3241-x", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1010431877", 
              "https://doi.org/10.1245/s10434-013-3241-x"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1007/s00401-019-01968-3", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1111894895", 
              "https://doi.org/10.1007/s00401-019-01968-3"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1245/s10434-017-6092-z", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1092238631", 
              "https://doi.org/10.1245/s10434-017-6092-z"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/modpathol.2012.83", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1021050243", 
              "https://doi.org/10.1038/modpathol.2012.83"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/modpathol.2017.54", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1090853041", 
              "https://doi.org/10.1038/modpathol.2017.54"
            ], 
            "type": "CreativeWork"
          }
        ], 
        "datePublished": "2019-08-28", 
        "datePublishedReg": "2019-08-28", 
        "description": "Solitary fibrous tumor is a rare mesenchymal neoplasm that exhibits a broad spectrum of biological behaviors. Few studies relative to clinical-pathologic features and predictive factors have been reported, all involving a mixed population of tumors occurring at different anatomic sites. In this study, we described a cohort of 41 patients with solitary fibrous tumor of the extremities and evaluated the prognostic role of clinical and histological features, presence of C228T and C250T mutations at the TERT promoter region, and NAB2\u2013STAT6 fusion variants. Patients were stratified according to the latest risk stratification model proposed by Demicco. The two patients with metastasis at presentation were in the high-risk group; the one with metastasis after surgery was classified in the intermediate-risk group. TERT promoter mutations were detected in 9 out of 38 DNA available. All patients with metastasis were characterized by a TERT promoter mutation. TERT promoter mutation was associated with mitoses >\u20094 per high-power field (p\u2009=\u20090.001), necrosis (p\u2009=\u20090.049), and size >\u200910\u00a0cm (p\u2009=\u20090.031). NAB2\u2013STAT6 fusion variants were detected in 27 out of 41 cases without any prognostic value. In conclusion, we confirmed that the patients with solitary fibrous tumor of the limbs have a better prognosis than other solitary fibrous tumors, with a very low percentage of metastatic events. Besides, our data support an association between TERT promoter mutations and histologically malignant features, suggesting a possible molecular role in stratifying patients into intermediate- to high-risk tumor.", 
        "genre": "article", 
        "id": "sg:pub.10.1007/s00428-019-02650-5", 
        "isAccessibleForFree": false, 
        "isPartOf": [
          {
            "id": "sg:journal.1106235", 
            "issn": [
              "0945-6317", 
              "1432-2307"
            ], 
            "name": "Virchows Archiv", 
            "publisher": "Springer Nature", 
            "type": "Periodical"
          }, 
          {
            "issueNumber": "3", 
            "type": "PublicationIssue"
          }, 
          {
            "type": "PublicationVolume", 
            "volumeNumber": "476"
          }
        ], 
        "keywords": [
          "solitary fibrous tumor", 
          "TERT promoter mutations", 
          "NAB2-STAT6 fusion variants", 
          "fibrous tumor", 
          "promoter mutations", 
          "clinical-pathologic features", 
          "intermediate-risk group", 
          "fusion variants", 
          "high-risk tumors", 
          "risk stratification model", 
          "high-risk group", 
          "rare mesenchymal neoplasm", 
          "high-power field", 
          "different anatomic sites", 
          "C250T mutations", 
          "prognostic role", 
          "TERT promoter region", 
          "better prognosis", 
          "predictive factors", 
          "prognostic value", 
          "histological features", 
          "mesenchymal neoplasms", 
          "malignant features", 
          "clinical correlation", 
          "anatomic sites", 
          "patients", 
          "metastatic events", 
          "C228T", 
          "tumors", 
          "stratification model", 
          "metastasis", 
          "biological behavior", 
          "possible molecular role", 
          "T mutation", 
          "molecular features", 
          "extremities", 
          "lower percentage", 
          "mutations", 
          "broad spectrum", 
          "molecular role", 
          "surgery", 
          "prognosis", 
          "group", 
          "neoplasms", 
          "mixed population", 
          "cohort", 
          "necrosis", 
          "promoter region", 
          "limb", 
          "mitoses", 
          "presentation", 
          "variants", 
          "study", 
          "association", 
          "role", 
          "conclusion", 
          "population", 
          "percentage", 
          "factors", 
          "features", 
          "cases", 
          "events", 
          "correlation", 
          "presence", 
          "DNA", 
          "data", 
          "sites", 
          "values", 
          "region", 
          "size", 
          "model", 
          "behavior", 
          "intermediates", 
          "spectra", 
          "field"
        ], 
        "name": "Histological and molecular features of solitary fibrous tumor of the extremities: clinical correlation", 
        "pagination": "445-454", 
        "productId": [
          {
            "name": "dimensions_id", 
            "type": "PropertyValue", 
            "value": [
              "pub.1120656632"
            ]
          }, 
          {
            "name": "doi", 
            "type": "PropertyValue", 
            "value": [
              "10.1007/s00428-019-02650-5"
            ]
          }, 
          {
            "name": "pubmed_id", 
            "type": "PropertyValue", 
            "value": [
              "31463729"
            ]
          }
        ], 
        "sameAs": [
          "https://doi.org/10.1007/s00428-019-02650-5", 
          "https://app.dimensions.ai/details/publication/pub.1120656632"
        ], 
        "sdDataset": "articles", 
        "sdDatePublished": "2022-10-01T06:45", 
        "sdLicense": "https://scigraph.springernature.com/explorer/license/", 
        "sdPublisher": {
          "name": "Springer Nature - SN SciGraph project", 
          "type": "Organization"
        }, 
        "sdSource": "s3://com-springernature-scigraph/baseset/20221001/entities/gbq_results/article/article_806.jsonl", 
        "type": "ScholarlyArticle", 
        "url": "https://doi.org/10.1007/s00428-019-02650-5"
      }
    ]
     

    Download the RDF metadata as:  json-ld nt turtle xml License info

    HOW TO GET THIS DATA PROGRAMMATICALLY:

    JSON-LD is a popular format for linked data which is fully compatible with JSON.

    curl -H 'Accept: application/ld+json' 'https://scigraph.springernature.com/pub.10.1007/s00428-019-02650-5'

    N-Triples is a line-based linked data format ideal for batch operations.

    curl -H 'Accept: application/n-triples' 'https://scigraph.springernature.com/pub.10.1007/s00428-019-02650-5'

    Turtle is a human-readable linked data format.

    curl -H 'Accept: text/turtle' 'https://scigraph.springernature.com/pub.10.1007/s00428-019-02650-5'

    RDF/XML is a standard XML format for linked data.

    curl -H 'Accept: application/rdf+xml' 'https://scigraph.springernature.com/pub.10.1007/s00428-019-02650-5'


     

    This table displays all metadata directly associated to this object as RDF triples.

    304 TRIPLES      21 PREDICATES      125 URIs      105 LITERALS      20 BLANK NODES

    Subject Predicate Object
    1 sg:pub.10.1007/s00428-019-02650-5 schema:about N09b762ed138d49f78aae6c032175afd0
    2 N1a61953b0b3f4f26a14a463b79cbf3b9
    3 N462cc2c516404521a2b4e93061833bc2
    4 N5e03f8bf617548e58361ece4d4c5c4e3
    5 N872e0f3bc9704ef49ba0c9c2f39be7af
    6 Na63396c198094a5497c0362defa230ef
    7 Naf53a08be2e9409d96f3d9377d08f359
    8 Nc535dd99e9b14a2fa737f2b077469b39
    9 Nd7504ea0ac7a489898dd43cba169092d
    10 Nde5ada0ac702411abadba8a4e7b66f24
    11 Ndfe7890411d144dea66ceb0964d2eb4c
    12 Nf4a52ed137eb4d71b3b21d1210956ce9
    13 Nf9ccfabb889247faa1496591788b3962
    14 anzsrc-for:11
    15 anzsrc-for:1112
    16 schema:author Nae3e6d3aff0f4f26902e0d4182ff90de
    17 schema:citation sg:pub.10.1007/s00256-010-1032-z
    18 sg:pub.10.1007/s00401-019-01968-3
    19 sg:pub.10.1038/modpathol.2012.83
    20 sg:pub.10.1038/modpathol.2015.90
    21 sg:pub.10.1038/modpathol.2016.126
    22 sg:pub.10.1038/modpathol.2017.54
    23 sg:pub.10.1038/ng.2509
    24 sg:pub.10.1038/ng.2522
    25 sg:pub.10.1186/1756-9966-33-33
    26 sg:pub.10.1186/s13000-014-0224-6
    27 sg:pub.10.1245/s10434-013-3241-x
    28 sg:pub.10.1245/s10434-017-6092-z
    29 schema:datePublished 2019-08-28
    30 schema:datePublishedReg 2019-08-28
    31 schema:description Solitary fibrous tumor is a rare mesenchymal neoplasm that exhibits a broad spectrum of biological behaviors. Few studies relative to clinical-pathologic features and predictive factors have been reported, all involving a mixed population of tumors occurring at different anatomic sites. In this study, we described a cohort of 41 patients with solitary fibrous tumor of the extremities and evaluated the prognostic role of clinical and histological features, presence of C228T and C250T mutations at the TERT promoter region, and NAB2–STAT6 fusion variants. Patients were stratified according to the latest risk stratification model proposed by Demicco. The two patients with metastasis at presentation were in the high-risk group; the one with metastasis after surgery was classified in the intermediate-risk group. TERT promoter mutations were detected in 9 out of 38 DNA available. All patients with metastasis were characterized by a TERT promoter mutation. TERT promoter mutation was associated with mitoses > 4 per high-power field (p = 0.001), necrosis (p = 0.049), and size > 10 cm (p = 0.031). NAB2–STAT6 fusion variants were detected in 27 out of 41 cases without any prognostic value. In conclusion, we confirmed that the patients with solitary fibrous tumor of the limbs have a better prognosis than other solitary fibrous tumors, with a very low percentage of metastatic events. Besides, our data support an association between TERT promoter mutations and histologically malignant features, suggesting a possible molecular role in stratifying patients into intermediate- to high-risk tumor.
    32 schema:genre article
    33 schema:isAccessibleForFree false
    34 schema:isPartOf N708797bbc1654fde99478bc99bc1d1a7
    35 Ne3c8778d1e14480ca0e62131627c9cd3
    36 sg:journal.1106235
    37 schema:keywords C228T
    38 C250T mutations
    39 DNA
    40 NAB2-STAT6 fusion variants
    41 T mutation
    42 TERT promoter mutations
    43 TERT promoter region
    44 anatomic sites
    45 association
    46 behavior
    47 better prognosis
    48 biological behavior
    49 broad spectrum
    50 cases
    51 clinical correlation
    52 clinical-pathologic features
    53 cohort
    54 conclusion
    55 correlation
    56 data
    57 different anatomic sites
    58 events
    59 extremities
    60 factors
    61 features
    62 fibrous tumor
    63 field
    64 fusion variants
    65 group
    66 high-power field
    67 high-risk group
    68 high-risk tumors
    69 histological features
    70 intermediate-risk group
    71 intermediates
    72 limb
    73 lower percentage
    74 malignant features
    75 mesenchymal neoplasms
    76 metastasis
    77 metastatic events
    78 mitoses
    79 mixed population
    80 model
    81 molecular features
    82 molecular role
    83 mutations
    84 necrosis
    85 neoplasms
    86 patients
    87 percentage
    88 population
    89 possible molecular role
    90 predictive factors
    91 presence
    92 presentation
    93 prognosis
    94 prognostic role
    95 prognostic value
    96 promoter mutations
    97 promoter region
    98 rare mesenchymal neoplasm
    99 region
    100 risk stratification model
    101 role
    102 sites
    103 size
    104 solitary fibrous tumor
    105 spectra
    106 stratification model
    107 study
    108 surgery
    109 tumors
    110 values
    111 variants
    112 schema:name Histological and molecular features of solitary fibrous tumor of the extremities: clinical correlation
    113 schema:pagination 445-454
    114 schema:productId N4b089a392a95428bbab88f24a0e58c60
    115 Nb53109ceba3044d98190ebaa0dad86f2
    116 Ndd007b3abcaa46aeb8c35b3bfbb1b817
    117 schema:sameAs https://app.dimensions.ai/details/publication/pub.1120656632
    118 https://doi.org/10.1007/s00428-019-02650-5
    119 schema:sdDatePublished 2022-10-01T06:45
    120 schema:sdLicense https://scigraph.springernature.com/explorer/license/
    121 schema:sdPublisher N1f38d98be593452da1b05ff7f330b256
    122 schema:url https://doi.org/10.1007/s00428-019-02650-5
    123 sgo:license sg:explorer/license/
    124 sgo:sdDataset articles
    125 rdf:type schema:ScholarlyArticle
    126 N09b762ed138d49f78aae6c032175afd0 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    127 schema:name Adolescent
    128 rdf:type schema:DefinedTerm
    129 N11141d20a08947f5b1cccbc2cd806312 rdf:first sg:person.01215431251.01
    130 rdf:rest Nec4f4009282349b4a4dd405925ecf498
    131 N1a61953b0b3f4f26a14a463b79cbf3b9 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    132 schema:name Young Adult
    133 rdf:type schema:DefinedTerm
    134 N1c03049c28e54d68bad4688865a85282 rdf:first sg:person.01202643430.41
    135 rdf:rest N11141d20a08947f5b1cccbc2cd806312
    136 N1f38d98be593452da1b05ff7f330b256 schema:name Springer Nature - SN SciGraph project
    137 rdf:type schema:Organization
    138 N22dada837d0b439d95a7ce9cc1a6e5c3 rdf:first sg:person.0746240275.84
    139 rdf:rest Nc489635d8a8e420ea96061bb3635c864
    140 N462cc2c516404521a2b4e93061833bc2 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    141 schema:name Aged
    142 rdf:type schema:DefinedTerm
    143 N4b089a392a95428bbab88f24a0e58c60 schema:name doi
    144 schema:value 10.1007/s00428-019-02650-5
    145 rdf:type schema:PropertyValue
    146 N5e03f8bf617548e58361ece4d4c5c4e3 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    147 schema:name Adult
    148 rdf:type schema:DefinedTerm
    149 N708797bbc1654fde99478bc99bc1d1a7 schema:volumeNumber 476
    150 rdf:type schema:PublicationVolume
    151 N75ba042862f343d59dedfd7ac8dfc477 rdf:first sg:person.010507452341.44
    152 rdf:rest N1c03049c28e54d68bad4688865a85282
    153 N7e9ed661fc1b4dd9b4e530d66cca0349 rdf:first sg:person.01031543327.58
    154 rdf:rest N22dada837d0b439d95a7ce9cc1a6e5c3
    155 N842ef7dfdc694eacbb5786d6c71ae416 rdf:first sg:person.01021210240.50
    156 rdf:rest N7e9ed661fc1b4dd9b4e530d66cca0349
    157 N872e0f3bc9704ef49ba0c9c2f39be7af schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    158 schema:name Male
    159 rdf:type schema:DefinedTerm
    160 Na63396c198094a5497c0362defa230ef schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    161 schema:name Humans
    162 rdf:type schema:DefinedTerm
    163 Nae3e6d3aff0f4f26902e0d4182ff90de rdf:first sg:person.016345573425.18
    164 rdf:rest N842ef7dfdc694eacbb5786d6c71ae416
    165 Naf53a08be2e9409d96f3d9377d08f359 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    166 schema:name Solitary Fibrous Tumors
    167 rdf:type schema:DefinedTerm
    168 Nb53109ceba3044d98190ebaa0dad86f2 schema:name pubmed_id
    169 schema:value 31463729
    170 rdf:type schema:PropertyValue
    171 Nc489635d8a8e420ea96061bb3635c864 rdf:first sg:person.01102031714.37
    172 rdf:rest N75ba042862f343d59dedfd7ac8dfc477
    173 Nc535dd99e9b14a2fa737f2b077469b39 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    174 schema:name Telomerase
    175 rdf:type schema:DefinedTerm
    176 Nd7504ea0ac7a489898dd43cba169092d schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    177 schema:name Middle Aged
    178 rdf:type schema:DefinedTerm
    179 Ndd007b3abcaa46aeb8c35b3bfbb1b817 schema:name dimensions_id
    180 schema:value pub.1120656632
    181 rdf:type schema:PropertyValue
    182 Nde5ada0ac702411abadba8a4e7b66f24 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    183 schema:name Extremities
    184 rdf:type schema:DefinedTerm
    185 Ndfe7890411d144dea66ceb0964d2eb4c schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    186 schema:name Female
    187 rdf:type schema:DefinedTerm
    188 Ne3c8778d1e14480ca0e62131627c9cd3 schema:issueNumber 3
    189 rdf:type schema:PublicationIssue
    190 Nec4f4009282349b4a4dd405925ecf498 rdf:first sg:person.01115347567.94
    191 rdf:rest rdf:nil
    192 Nf4a52ed137eb4d71b3b21d1210956ce9 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    193 schema:name Mutation
    194 rdf:type schema:DefinedTerm
    195 Nf9ccfabb889247faa1496591788b3962 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    196 schema:name Aged, 80 and over
    197 rdf:type schema:DefinedTerm
    198 anzsrc-for:11 schema:inDefinedTermSet anzsrc-for:
    199 schema:name Medical and Health Sciences
    200 rdf:type schema:DefinedTerm
    201 anzsrc-for:1112 schema:inDefinedTermSet anzsrc-for:
    202 schema:name Oncology and Carcinogenesis
    203 rdf:type schema:DefinedTerm
    204 sg:journal.1106235 schema:issn 0945-6317
    205 1432-2307
    206 schema:name Virchows Archiv
    207 schema:publisher Springer Nature
    208 rdf:type schema:Periodical
    209 sg:person.01021210240.50 schema:affiliation grid-institutes:grid.419038.7
    210 schema:familyName Sambri
    211 schema:givenName Andrea
    212 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01021210240.50
    213 rdf:type schema:Person
    214 sg:person.01031543327.58 schema:affiliation grid-institutes:grid.419038.7
    215 schema:familyName Pedrini
    216 schema:givenName Elena
    217 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01031543327.58
    218 rdf:type schema:Person
    219 sg:person.010507452341.44 schema:affiliation grid-institutes:grid.412029.c
    220 schema:familyName Ruengwanichayakun
    221 schema:givenName Poosit
    222 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.010507452341.44
    223 rdf:type schema:Person
    224 sg:person.01102031714.37 schema:affiliation grid-institutes:grid.419038.7
    225 schema:familyName Sangiorgi
    226 schema:givenName Luca
    227 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01102031714.37
    228 rdf:type schema:Person
    229 sg:person.01115347567.94 schema:affiliation grid-institutes:grid.419038.7
    230 schema:familyName Righi
    231 schema:givenName Alberto
    232 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01115347567.94
    233 rdf:type schema:Person
    234 sg:person.01202643430.41 schema:affiliation grid-institutes:grid.419038.7
    235 schema:familyName Donati
    236 schema:givenName Davide
    237 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01202643430.41
    238 rdf:type schema:Person
    239 sg:person.01215431251.01 schema:affiliation grid-institutes:grid.419038.7
    240 schema:familyName Benassi
    241 schema:givenName Maria Serena
    242 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01215431251.01
    243 rdf:type schema:Person
    244 sg:person.016345573425.18 schema:affiliation grid-institutes:grid.419038.7
    245 schema:familyName Bianchi
    246 schema:givenName Giuseppe
    247 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.016345573425.18
    248 rdf:type schema:Person
    249 sg:person.0746240275.84 schema:affiliation grid-institutes:grid.419038.7
    250 schema:familyName Pazzaglia
    251 schema:givenName Laura
    252 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0746240275.84
    253 rdf:type schema:Person
    254 sg:pub.10.1007/s00256-010-1032-z schema:sameAs https://app.dimensions.ai/details/publication/pub.1033624330
    255 https://doi.org/10.1007/s00256-010-1032-z
    256 rdf:type schema:CreativeWork
    257 sg:pub.10.1007/s00401-019-01968-3 schema:sameAs https://app.dimensions.ai/details/publication/pub.1111894895
    258 https://doi.org/10.1007/s00401-019-01968-3
    259 rdf:type schema:CreativeWork
    260 sg:pub.10.1038/modpathol.2012.83 schema:sameAs https://app.dimensions.ai/details/publication/pub.1021050243
    261 https://doi.org/10.1038/modpathol.2012.83
    262 rdf:type schema:CreativeWork
    263 sg:pub.10.1038/modpathol.2015.90 schema:sameAs https://app.dimensions.ai/details/publication/pub.1035569641
    264 https://doi.org/10.1038/modpathol.2015.90
    265 rdf:type schema:CreativeWork
    266 sg:pub.10.1038/modpathol.2016.126 schema:sameAs https://app.dimensions.ai/details/publication/pub.1003285631
    267 https://doi.org/10.1038/modpathol.2016.126
    268 rdf:type schema:CreativeWork
    269 sg:pub.10.1038/modpathol.2017.54 schema:sameAs https://app.dimensions.ai/details/publication/pub.1090853041
    270 https://doi.org/10.1038/modpathol.2017.54
    271 rdf:type schema:CreativeWork
    272 sg:pub.10.1038/ng.2509 schema:sameAs https://app.dimensions.ai/details/publication/pub.1049090988
    273 https://doi.org/10.1038/ng.2509
    274 rdf:type schema:CreativeWork
    275 sg:pub.10.1038/ng.2522 schema:sameAs https://app.dimensions.ai/details/publication/pub.1052144262
    276 https://doi.org/10.1038/ng.2522
    277 rdf:type schema:CreativeWork
    278 sg:pub.10.1186/1756-9966-33-33 schema:sameAs https://app.dimensions.ai/details/publication/pub.1015633596
    279 https://doi.org/10.1186/1756-9966-33-33
    280 rdf:type schema:CreativeWork
    281 sg:pub.10.1186/s13000-014-0224-6 schema:sameAs https://app.dimensions.ai/details/publication/pub.1018093528
    282 https://doi.org/10.1186/s13000-014-0224-6
    283 rdf:type schema:CreativeWork
    284 sg:pub.10.1245/s10434-013-3241-x schema:sameAs https://app.dimensions.ai/details/publication/pub.1010431877
    285 https://doi.org/10.1245/s10434-013-3241-x
    286 rdf:type schema:CreativeWork
    287 sg:pub.10.1245/s10434-017-6092-z schema:sameAs https://app.dimensions.ai/details/publication/pub.1092238631
    288 https://doi.org/10.1245/s10434-017-6092-z
    289 rdf:type schema:CreativeWork
    290 grid-institutes:grid.412029.c schema:alternateName Department of Pathology, Faculty of Medicine, Naresuan University, Phitsanulok, Thailand
    291 schema:name Department of Pathology, Faculty of Medicine, Naresuan University, Phitsanulok, Thailand
    292 Department of Pathology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
    293 rdf:type schema:Organization
    294 grid-institutes:grid.419038.7 schema:alternateName Department of Medical Genetics and Rare Orthopedic Diseases and CLIBI Laboratory, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
    295 Department of Medical Genetics and Rare Orthopedic Diseases, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
    296 Department of Orthopedic Oncology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
    297 Department of Pathology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
    298 Experimental Oncology Laboratory, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
    299 schema:name Department of Medical Genetics and Rare Orthopedic Diseases and CLIBI Laboratory, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
    300 Department of Medical Genetics and Rare Orthopedic Diseases, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
    301 Department of Orthopedic Oncology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
    302 Department of Pathology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
    303 Experimental Oncology Laboratory, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
    304 rdf:type schema:Organization
     




    Preview window. Press ESC to close (or click here)


    ...