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Solitary fibrous tumour: Strategies for risk prognostication and management.

Solitary fibrous tumours (SFT) are uncommon mesenchymal tumours characterized by NAB2-STAT6 gene fusions. While historical models classified SFT as benign or malignant based on histologic features, more recent risk stratification models have been proposed to replace this classification and improve r...

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Published in:Canadian Journal of Pathology Vol. 11; no. 1; pp. 70 - 81
Main Author: Demicco, Elizabeth G.
Format: Journal Article
Published: Canadian Association of Pathologists Feb2019
Online Access:View this record in EBSCOhost
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      jtl: Canadian Journal of Pathology
      issn: 1918915X
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      dt: Feb2019
      vid: 11
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      pub: Canadian Association of Pathologists
      place: Kingston, Ontario
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        atl: Solitary fibrous tumour: Strategies for risk prognostication and management.
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        au: Demicco, Elizabeth G.
        affil: Staff Pathologist, Department of Pathology and Laboratory Medicine, Mount Sinai Hospital, Toronto, ON, Canada
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        Solitary fibrous tumours (SFT) are uncommon mesenchymal tumours characterized by NAB2-STAT6 gene fusions. While historical models classified SFT as benign or malignant based on histologic features, more recent risk stratification models have been proposed to replace this classification and improve risk prediction and patient management. The discovery of the molecular driver of tumourigenesis - the NAB2-STAT6 fusion gene - has further improved diagnostic accuracy and may impact future patient management. This review provides an update on clinicopathologic risk models for SFT and recent advances in understanding SFT biology.
        Les tumeurs fibreuses solitaires (TFS) sont des tumeurs mésenchymateuses rares caractérisées par la présence du gène de fusion NAB2-STAT6. Si, à l'origine, les TFS étaient qualifiées de bénignes ou de malignes en fonction de leurs caractéristiques histologiques, récemment, des modèles de stratification du risque ont été proposés pour remplacer cette classification et améliorer la prévision du risque et la prise en charge des patients. De plus, la découverte du mécanisme moléculaire sousjacent à la tumorigénèse - le gène de fusion NAB2-STAT6 - a permis d'améliorer encore davantage l'exactitude diagnostique et pourrait avoir des répercussions positives sur la prise en charge future des patients. Cet article rend compte des dernières mises à jour associées aux modèles de risque clinicopathologique des TFS et des plus récents progrès dans la compréhension de la biologie de ces tumeurs.
      pubtype: Academic Journal
      doctype: Journal Article
      ougenre: Article
    language: Multiple languages
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