Skip to main content

Solitary Fibrous Tumor, Soft Tissue

  • Living reference work entry
  • First Online:
Encyclopedia of Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

  • 31 Accesses

Synonyms

Giant cell angiofibroma; Hemangiopericytoma; Lipomatous solitary fibrous tumor

Definition

A mesenchymal tumor showing fibroblastic differentiation, prominent branching vasculature, and characterized by recurrent NGFI-A binding protein 2-signal transducer and activator of transcription 6 (NAB2-STAT6) gene fusions and variable clinical course.

Clinical Features

  • Incidence

    The true incidence of soft tissue solitary fibrous tumors (SFT) is unknown, but they are thought to comprise approximately 2–3% of all soft tissue tumors, with so-called “malignant” SFT accounting for 0.8% of soft tissue tumors (Gold et al. 2002; Penel et al. 2018).

  • Age

    Soft tissue SFT present with a peak incidence between 40 and 70 years. However, tumors may arise at any age, including the pediatric population.

  • Sex

    There is an equal sex distribution for soft tissue SFT.

  • Site

    SFT can arise at any anatomic site but demonstrate an apparent predilection for the soft tissue of serosalized cavities (pleura and...

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Institutional subscriptions

References

  • Akaike, K., Kurisaki-Arakawa, A., Hara, K., Suehara, Y., Takagi, T., Mitani, K., Kaneko, K., Yao, T., & Saito, T. (2015a). Distinct clinicopathological features of NAB2-STAT6 fusion gene variants in solitary fibrous tumor with emphasis on the acquisition of highly malignant potential. Human Pathology, 46, 347–356.

    Article  CAS  Google Scholar 

  • Akaike, K., Toda-Ishii, M., Suehara, Y., Mukaihara, K., Kubota, D., Mitani, K., Takagi, T., Kaneko, K., Yao, T., & Saito, T. (2015b). TERT promoter mutations are a rare event in gastrointestinal stromal tumors. Springerplus, 4, 836.

    Article  Google Scholar 

  • Bahrami, A., Lee, S., Schaefer, I. M., Boland, J. M., Patton, K. T., Pounds, S., & Fletcher, C. D. (2016). TERT promoter mutations and prognosis in solitary fibrous tumor. Modern Pathology, 29, 1511–1522.

    Article  CAS  Google Scholar 

  • Barthelmeẞ, S., Geddert, H., Boltze, C., Moskalev, E. A., Bieg, M., Sirbu, H., Brors, B., Wiemann, S., Hartmann, A., Agaimy, A., et al. (2014). Solitary fibrous tumors/hemangiopericytomas with different variants of the NAB2-STAT6 gene fusion are characterized by specific histomorphology and distinct clinicopathological features. The American Journal of Pathology, 184, 1209–1218.

    Article  Google Scholar 

  • Bertucci, F., Bouvier-Labit, C., Finetti, P., Metellus, P., Adelaide, J., Mokhtari, K., Figarella-Branger, D., Decouvelaere, A. V., Miquel, C., Coindre, J. M., et al. (2013). Gene expression profiling of solitary fibrous tumors. PLoS One, 8, e64497.

    Article  CAS  Google Scholar 

  • Bishop, A. J., Zagars, G. K., Demicco, E. G., Wang, W. L., Feig, B. W., & Guadagnolo, B. A. (2018). Soft tissue solitary fibrous tumor: Combined surgery and radiation therapy results in excellent local control. American Journal of Clinical Oncology, 41, 81–85.

    Article  Google Scholar 

  • Bouvier, C., Bertucci, F., Metellus, P., Finetti, P., Maues de Paula, A., Forest, F., Mokhtari, K., Miquel, C., Birnbaum, D., Vasiljevic, A., et al. (2013). ALDH1 is an immunohistochemical diagnostic marker for solitary fibrous tumours and haemangiopericytomas of the meninges emerging from gene profiling study. Acta Neuropathologica Communications, 1, 10.

    Article  Google Scholar 

  • Chmielecki, J., Crago, A. M., Rosenberg, M., O’Connor, R., Walker, S. R., Ambrogio, L., Auclair, D., McKenna, A., Heinrich, M. C., Frank, D. A., et al. (2013). Whole-exome sequencing identifies a recurrent NAB2-STAT6 fusion in solitary fibrous tumors. Nature Genetics, 45, 131–132.

    Article  CAS  Google Scholar 

  • Collini, P., Negri, T., Barisella, M., Palassini, E., Tarantino, E., Pastorino, U., Gronchi, A., Stacchiotti, S., & Pilotti, S. (2012). High-grade sarcomatous overgrowth in solitary fibrous tumors: A clinicopathologic study of 10 cases. The American Journal of Surgical Pathology, 36, 1202–1215.

    Article  Google Scholar 

  • Dagrada, G. P., Spagnuolo, R. D., Mauro, V., Tamborini, E., Cesana, L., Gronchi, A., Stacchiotti, S., Pierotti, M. A., Negri, T., & Pilotti, S. (2015). Solitary fibrous tumors: Loss of chimeric protein expression and genomic instability mark dedifferentiation. Modern Pathology, 28, 1074–1083.

    Article  CAS  Google Scholar 

  • de Perrot, M., Fischer, S., Brundler, M. A., Sekine, Y., & Keshavjee, S. (2002). Solitary fibrous tumors of the pleura. The Annals of Thoracic Surgery, 74, 285–293.

    Article  Google Scholar 

  • Dei Tos, A. P., Seregard, S., Calonje, E., Chan, J. K., & Fletcher, C. D. (1995). Giant cell angiofibroma. A distinctive orbital tumor in adults. The American Journal of Surgical Pathology, 19, 1286–1293.

    Article  CAS  Google Scholar 

  • Demicco, E. G., Wagner, M. J., Maki, R. G., Gupta, V., Iofin, I., Lazar, A. J., & Wang, W. L. (2017). Risk assessment in solitary fibrous tumors: Validation and refinement of a risk stratification model. Modern Pathology, 30, 1433–1442.

    Article  Google Scholar 

  • Demicco, E. G., Wani, K., Ingram, D., Wagner, M., Maki, R. G., Rizzo, A., Meeker, A., Lazar, A. J., & Wang, W. L. (2018). TERT promoter mutations in solitary fibrous tumor. Histopathology, 73, 843–851.

    Article  Google Scholar 

  • Demicco, E. G., Griffin, A. M., Gladdy, R. A., Dickson, B. C., Ferguson, P. C., Swallow, C. J., Wunder, J. S., & Wang, W. L. (2019). Comparison of published risk models for prediction of outcome in patients with extrameningeal solitary fibrous tumour. Histopathology, 75, 723–737.

    Article  Google Scholar 

  • Diebold, M., Soltermann, A., Hottinger, S., Haile, S. R., Bubendorf, L., Komminoth, P., Jochum, W., Grobholz, R., Theegarten, D., Berezowska, S., et al. (2017). Prognostic value of MIB-1 proliferation index in solitary fibrous tumors of the pleura implemented in a new score – A multicenter study. Respiratory Research, 18, 210.

    Article  Google Scholar 

  • Doyle, L. A., Vivero, M., Fletcher, C. D., Mertens, F., & Hornick, J. L. (2014). Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics. Modern Pathology, 27, 390–395.

    Article  CAS  Google Scholar 

  • England, D. M., Hochholzer, L., & McCarthy, M. J. (1989). Localized benign and malignant fibrous tumors of the pleura. A clinicopathologic review of 223 cases. The American Journal of Surgical Pathology, 13, 640–658.

    Article  CAS  Google Scholar 

  • Fletcher, C. D. M., Bridge, J. A., & Lee, J.-C. (2013). Extrapleural solitary fibrous tumor. In C. D. M. Fletcher, J. A. Bridge, P. C. W. Hogendoorn, & F. Mertens (Eds.), World Health Organization of tumors of soft tissue and bone (pp. 80–82). Lyon: IARC Press.

    Google Scholar 

  • Folpe, A. L., Devaney, K., & Weiss, S. W. (1999). Lipomatous hemangiopericytoma: A rare variant of hemangiopericytoma that may be confused with liposarcoma. The American Journal of Surgical Pathology, 23, 1201–1207.

    Article  CAS  Google Scholar 

  • Friis, R. B., Safwat, A., Baad-Hansen, T., & Aggerholm-Pedersen, N. (2018). Solitary fibrous tumour: A single institution retrospective study and further validation of a prognostic risk assessment system. Clinical Oncology (Royal College of Radiologists), 30, 798–804.

    Article  CAS  Google Scholar 

  • Gold, J. S., Antonescu, C. R., Hajdu, C., Ferrone, C. R., Hussain, M., Lewis, J. J., Brennan, M. F., & Coit, D. G. (2002). Clinicopathologic correlates of solitary fibrous tumors. Cancer, 94, 1057–1068.

    Article  Google Scholar 

  • Guillou, L., Gebhard, S., & Coindre, J. M. (2000a). Lipomatous hemangiopericytoma: A fat-containing variant of solitary fibrous tumor? Clinicopathologic, immunohistochemical, and ultrastructural analysis of a series in favor of a unifying concept. Human Pathology, 31, 1108–1115.

    Article  CAS  Google Scholar 

  • Guillou, L., Gebhard, S., & Coindre, J. M. (2000b). Orbital and extraorbital giant cell angiofibroma: A giant cell-rich variant of solitary fibrous tumor? Clinicopathologic and immunohistochemical analysis of a series in favor of a unifying concept. The American Journal of Surgical Pathology, 24, 971–979.

    Article  CAS  Google Scholar 

  • Hajdu, M., Singer, S., Maki, R. G., Schwartz, G. K., Keohan, M. L., & Antonescu, C. R. (2010). IGF2 over-expression in solitary fibrous tumours is independent of anatomical location and is related to loss of imprinting. The Journal of Pathology, 221, 300–307.

    Article  CAS  Google Scholar 

  • Machado, I., Morales, G. N., Cruz, J., Lavernia, J., Giner, F., Navarro, S., Ferrandez, A., & Llombart-Bosch, A. (2019). Solitary fibrous tumor: A case series identifying pathological adverse factors-implications for risk stratification and classification. Virchows Archiv. https://doi.org/10.1007/s00428-019-02660-3.

  • Martin-Broto, J., Stacchiotti, S., Lopez-Pousa, A., Redondo, A., Bernabeu, D., de Alava, E., Casali, P. G., Italiano, A., Gutierrez, A., Moura, D. S., et al. (2019). Pazopanib for treatment of advanced malignant and dedifferentiated solitary fibrous tumour: A multicentre, single-arm, phase 2 trial. The Lancet Oncology, 20, 134–144.

    Article  CAS  Google Scholar 

  • Mohajeri, A., Tayebwa, J., Collin, A., Nilsson, J., Magnusson, L., von Steyern, F. V., Brosjo, O., Domanski, H. A., Larsson, O., Sciot, R., et al. (2013). Comprehensive genetic analysis identifies a pathognomonic NAB2/STAT6 fusion gene, nonrandom secondary genomic imbalances, and a characteristic gene expression profile in solitary fibrous tumor. Genes, Chromosomes & Cancer, 52, 873–886.

    Article  CAS  Google Scholar 

  • Mosquera, J. M., & Fletcher, C. D. (2009). Expanding the spectrum of malignant progression in solitary fibrous tumors: A study of 8 cases with a discrete anaplastic component–is this dedifferentiated SFT? The American Journal of Surgical Pathology, 33, 1314–1321.

    Article  Google Scholar 

  • Ng, D. W. J., Tan, G. H. C., Soon, J. J. Y., Zhao, D. Y., Shannon, N. B., Selvarajan, S., Soo, K. C., & Teo, M. C. C. (2018). The approach to solitary fibrous tumors: Are clinicopathological features and nomograms accurate in the prediction of prognosis? International Journal of Surgical Pathology, 26, 600–608.

    Article  Google Scholar 

  • Pasquali, S., Gronchi, A., Strauss, D., Bonvalot, S., Jeys, L., Stacchiotti, S., Hayes, A., Honore, C., Collini, P., Renne, S. L., et al. (2016). Resectable extra-pleural and extra-meningeal solitary fibrous tumours: A multi-centre prognostic study. European Journal of Surgical Oncology, 42, 1064–1070.

    Article  CAS  Google Scholar 

  • Penel, N., Coindre, J. M., Giraud, A., Terrier, P., Ranchere-Vince, D., Collin, F., Guellec, S. L. E., Bazille, C., Lae, M., de Pinieux, G., et al. (2018). Presentation and outcome of frequent and rare sarcoma histologic subtypes: A study of 10,262 patients with localized visceral/soft tissue sarcoma managed in reference centers. Cancer, 124, 1179–1187.

    Article  Google Scholar 

  • Reisenauer, J. S., Mneimneh, W., Jenkins, S., Mansfield, A. S., Aubry, M. C., Fritchie, K. J., Allen, M. S., Blackmon, S. H., Cassivi, S. D., Nichols, F. C., et al. (2018). Comparison of risk stratification models to predict recurrence and survival in pleuropulmonary solitary fibrous tumor. Journal of Thoracic Oncology, 13, 1349–1362.

    Article  Google Scholar 

  • Robinson, D. R., Wu, Y. M., Kalyana-Sundaram, S., Cao, X., Lonigro, R. J., Sung, Y. S., Chen, C. L., Zhang, L., Wang, R., Su, F., et al. (2013). Identification of recurrent NAB2-STAT6 gene fusions in solitary fibrous tumor by integrative sequencing. Nature Genetics, 45, 180–185.

    Article  CAS  Google Scholar 

  • Salas, S., Resseguier, N., Blay, J. Y., Le Cesne, A., Italiano, A., Chevreau, C., Rosset, P., Isambert, N., Soulie, P., Cupissol, D., et al. (2017). Prediction of local and metastatic recurrence in solitary fibrous tumor: Construction of a risk calculator in a multicenter cohort from the French sarcoma group (FSG) database. Annals of Oncology, 28, 1979–1987.

    Article  CAS  Google Scholar 

  • Schneider, N., Hallin, M., & Thway, K. (2017). STAT6 loss in dedifferentiated solitary fibrous tumor. International Journal of Surgical Pathology, 25, 58–60.

    Article  CAS  Google Scholar 

  • Smith, S. C., Gooding, W. E., Elkins, M., Patel, R. M., Harms, P. W., McDaniel, A. S., Palanisamy, N., Uram-Tuculescu, C., Balzer, B. B., Lucas, D. R., et al. (2017). Solitary fibrous tumors of the head and neck: A multi-institutional clinicopathologic study. The American Journal of Surgical Pathology, 41, 1642–1656.

    Article  Google Scholar 

  • Tapias, L. F., Mercier, O., Ghigna, M. R., Lahon, B., Lee, H., Mathisen, D. J., Dartevelle, P., & Lanuti, M. (2015). Validation of a scoring system to predict recurrence of resected solitary fibrous tumors of the pleura. Chest, 147, 216–223.

    Article  Google Scholar 

  • Thway, K., Hayes, A., Ieremia, E., & Fisher, C. (2013). Heterologous osteosarcomatous and rhabdomyosarcomatous elements in dedifferentiated solitary fibrous tumor: Further support for the concept of dedifferentiation in solitary fibrous tumor. Annals of Diagnostic Pathology, 17, 457–463.

    Article  Google Scholar 

  • Vivero, M., Doyle, L. A., Fletcher, C. D., Mertens, F., & Hornick, J. L. (2014). GRIA2 is a novel diagnostic marker for solitary fibrous tumour identified through gene expression profiling. Histopathology, 65, 71–80.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Elizabeth G. Demicco .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Demicco, E.G. (2020). Solitary Fibrous Tumor, Soft Tissue. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5490-1

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-28845-1_5490-1

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

  • eBook Packages: Springer Reference MedicineReference Module Medicine

Publish with us

Policies and ethics