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Solitary Fibrous Tumors/Hemangiopericytoma

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Radiation Therapy for Sarcomas and Skin Cancers

Part of the book series: Practical Guides in Radiation Oncology ((PGRO))

Abstract

Solitary fibrous tumors (SFTs) comprise a histologic spectrum of rare soft tissue neoplasms, demonstrating fibroblastic differentiation that includes hemangiopericytomas (HPCs). SFTs preferentially arise in serosal membranes, the dura of the meninges, and deep soft tissues. Identification of the NAB2-STAT6 fusion protein can aid in the diagnosis of SFTs, and this gene fusion is a defining feature of its co-classification with hemangiopericytomas. The majority of extracranial SFTs behave in an indolent fashion and do not recur locally or distantly; however, about 10–25 percent of tumors do behave more aggressively. Management of SFT should be discussed in a multidisciplinary tumor board with sarcoma specialists who have experience with the disease. Complete en bloc surgical resection with negative margins is the mainstay of primary therapy for all localized SFTs, given the low overall metastatic potential, and the use of adjuvant radiotherapy or chemotherapy for incompletely resected or recurrent SFTs is best decided on a case-by-case basis in the context of a multidisciplinary discussion. Patients with higher-risk features on final pathology tend to receive adjuvant radiotherapy in spite of a clear demonstrable survival benefit, and chemotherapy is generally reserved for treatment in the metastatic setting. Treatment should be followed by careful long-term postoperative surveillance, given the propensity for these tumors to spread distantly several years following therapy, and the frequency may be tailored to the risk of recurrence. SFTs can recur locally, and such recurrences can often be managed successfully with re-resection. For patients with metastatic and locally advanced unresectable tumors, traditional anthracycline-based soft tissue sarcoma chemotherapy has some efficacy, and pazopanib has been increasingly utilized. Intracranial HPCs are dural-based tumors and include a “classic” solitary fibrous tumor (SFT) phenotype corresponding to World Health Organization (WHO) grade I (minimal mitotic activity, ≤3 mitoses per 10 HPF) activity as well as a more biologically aggressive “hemangiopericytoma” histology (grade II–III). WHO grade II and III tumors (HPCs) have rapid growth and are considered biologically aggressive tumors, for which postoperative radiation is suggested in most patients. Even after gross total resection, regardless of grade, these tumors may recur locally, within the CNS, or extracranially, and retrospective data suggests that local control, disease-free survival, and overall survival rates all appear to be improved when patients receive adjuvant radiotherapy. Future directions for the management of these tumors include efforts at prospective study, incorporation of alternate modalities of radiotherapy, and better understanding both indications and optimal doses for radiotherapy, both as primary therapy and in the recurrent setting.

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Authors and Affiliations

  1. Department of Radiation Oncology, Northwestern University, Feinberg School of Medicine, Chicago, IL, USA

    Amishi Bajaj

  2. Department of Radiation Oncology, Lynn Cancer Institute - Boca Raton Regional Hospital, Boynton Beach, FL, USA

    Hina Saeed

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  1. Amishi Bajaj
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  2. Hina Saeed
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Correspondence to Hina Saeed .

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Editors and Affiliations

  1. Department of Radiation Oncology, University of Washington, Seattle, WA, USA

    Edward Kim

  2. Department of Radiation Oncology, University of Washington, Seattle, WA, USA

    Upendra Parvathaneni

  3. Department of Radiation Oncology, The Ohio State University James Cancer Center, Columbus, OH, USA

    Meng Xu Welliver

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Bajaj, A., Saeed, H. (2022). Solitary Fibrous Tumors/Hemangiopericytoma. In: Kim, E., Parvathaneni, U., Welliver, M.X. (eds) Radiation Therapy for Sarcomas and Skin Cancers. Practical Guides in Radiation Oncology. Springer, Cham. https://doi.org/10.1007/978-3-031-06706-8_9

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