Publication: Solitary fibrous tumor: An evolving and unifying entity with unsettled issues
Authors
Huang, Shih-Chiang ; Huang, Hsuan-Ying
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Publisher
Universidad de Murcia. Departamento de Biología Celular e Histología
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DOI
DOI: 10.14670/HH-18-064
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info:eu-repo/semantics/article
Description
Abstract
Solitary fibrous tumor (SFT) is a distinct
fibroblastic neoplasm of intermediate biological
potential, prototypically presenting as a pleuraassociated tumor characterized by patternless
proliferation of generally banal oval to spindle cells with
hemangiopericytoma-like staghorn vessels in
fibrocollagenous stroma. Over the past decades, the
clinicopathological spectrum of SFT has been everexpanding with the incorporation of cases exhibiting
myxoid, giant cell-containing, and fat-forming histology,
as well as those from extrathoracic sites, including the
meninx. Atypical, frankly malignant and even
dedifferentiated variants have also been recognized in a
subset of SFTs. Notably, the recent groundbreaking
discovery of the disease-defining NAB2-STAT6 gene
fusion, resulting from intrachromosomal inversion
involving 12q13.3, has largely unified tumors with the
aforementioned variations. The derived immunohistochemical detection of nuclear STAT6 expression has
high diagnostic value in distinguishing SFTs from
histologic mimics, although some relevant pitfalls have
been proposed as a precaution. NAB2-STAT6 fusions
yield numerous transcript subtypes associated with the
clinicopathological variations. Despite mostly following
a favorable course, SFT is notoriously difficult for
prognostication because of the propensity for late relapse
or even metastases in 10-40% of cases, which prompts
several proposed schemes incorporating age, size,
mitosis, and/or necrosis as factors for risk stratification.
Mitotic figures >4/10 HPFs, TERT promoter and/or
TP53 mutations have been considered as variables that
are better correlated with aggressiveness. Although
radiotherapy and chemotherapy provide unsatisfactory
responses, a better understanding of SFT tumorigenesis
may pave the way for new treatment modalities. In this
review, we comprehensively discuss the recent advances
of SFTs in diagnostic and molecular pathology.
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Citation
Histology and Histopathology, Vol.34, nº4, (2019)
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