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Solitary fibrous tumor: a case series identifying pathological adverse factors—implications for risk stratification and classification

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Abstract

Solitary fibrous tumors (SFTs) are a rare type of mesenchymal lesion in which specific clinicopathologic factors have been related to patient outcome. We collected clinical, pathological, and molecular data of 28 patients with histologically confirmed SFT having at least one pathological factor associated with aggressive behavior. Molecular analysis to detect NAB2/STAT6 gene fusion, TP53, and/or TERT promoter mutation was performed. We analyzed the pathological factors predictive of recurrence/metastasis and compared with clinical outcome. The risk of metastasis was calculated using four previously described scoring systems. Histopathologically, all tumors revealed hypercellularity, 11 had ≥ 4 mitoses/10 HPF, and 12 showed necrosis. Dedifferentiation was observed in three tumors. STAT6 was positive in all cases. Desmin, p16, INSM1, and HTER immunoexpressions were detected in 14, 18, 21, and 46% of the SFT, respectively. The NAB2/STAT6 gene fusion was detected in 16 tumors. After a median follow-up of 34 months, 32.0% recurred, 32.1% metastasized, and 35.7% died of disease. TERT mutations were detected in almost half the tumors. Tumors with TP53 mutations or with TP53 and TERT promoter mutations were almost always classified as high risk, and the patients developed metastases and/or died of disease. Tumors with intermediate-risk and TERT mutation had a worse evolution. SFTs with adverse pathological parameters were not always related with a poor outcome, thus confirming the unpredictable clinical behavior of SFT. The inclusion of molecular factors (TP53 and TERT promoter status) may provide new prognostic indicators for future risk stratification systems, especially in the intermediate-risk group.

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  • 26 October 2019

    In Tables 4 and 5, there were entries that were not properly aligned on its designated rows. The correct tables are presented in this article.]

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Acknowledgments

The authors thank David Harrison for the English review and editing of the manuscript. This study was supported in part by grant from Instituto Valenciano de Oncología.

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Authors and Affiliations

  1. Pathology Department, Instituto Valenciano de Oncología, Valencia, Spain

    Isidro Machado & Julia Cruz

  2. Molecular Biology, Pathology Department, University of Valencia, Valencia, Spain

    Gema Nieto Morales

  3. Department of Oncology, Instituto Valenciano de Oncología, Valencia, Spain

    Javier Lavernia

  4. Pathology Department, University Hospital “La Fe”, Valencia, Spain

    Francisco Giner

  5. Pathology Department, University of Valencia, Valencia, Spain

    Samuel Navarro, Antonio Ferrandez & Antonio Llombart-Bosch

Authors
  1. Isidro Machado
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  2. Gema Nieto Morales
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  8. Antonio Llombart-Bosch
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Correspondence to Isidro Machado.

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The present work is a retrospective study that fulfills the ethical requirements. There are no conflicts of interest.

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The authors declare that they have no conflict of interest.

Contributions

-Isidro Machado: study proposal, writing of the manuscript, collection of cases, immunohistochemical analysis, results analysis, final approval of the manuscript

-Gema Nieto Morales: molecular analysis, final approval of the manuscript

-Julia Cruz: study proposal, results analysis, final approval of the manuscript

-Javier Lavernia: collection of cases, contribution with the clinical variables of each case, final approval of the manuscript

-Francisco Giner: study proposal, results analysis, final approval of the manuscript

-Samuel Navarro: study proposal, results analysis, final approval of the manuscript

-Antonio Ferrandez: study proposal, results analysis, final approval of the manuscript

-Antonio Llombart-Bosch: study proposal, collection of cases, immunohistochemical analysis, results analysis, final approval of the manuscript

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The original version of this article was revised: The In Tables 4 and 5, there were entries that were not properly aligned on its designated rows. The correct tables are presented in this article.

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Machado, I., Morales, G.N., Cruz, J. et al. Solitary fibrous tumor: a case series identifying pathological adverse factors—implications for risk stratification and classification. Virchows Arch 476, 597–607 (2020). https://doi.org/10.1007/s00428-019-02660-3

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