Radiation Therapy as Sole Management for Solitary Fibrous Tumors (SFT): A Retrospective Study From the Global SFT Initiative in Collaboration With the Sarcoma Patients EuroNet

Haas, Rick L.; Walraven, Iris; Lecointe-Artzner, Estelle; Scholten, Astrid N.; Houdt, Winan J. van; Griffin, Anthony M.; Ferguson, Peter C.; Miah, Aisha; Zaidi, Shaine; DeLaney, Thomas F.; Chen, Yen‐Lin; Spałek, Mateusz; Krol, Augustinus D.G.; Moeri-Schimmel, Rieneke G.; Sande, Michiel A. J. van de; Sangalli, Claudia; Stacchiotti, Silvia

doi:10.1016/j.ijrobp.2018.04.024

Cited by 45 publications

(29 citation statements)

References 45 publications

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“…In an analysis of 155 patients with central nervous system SFTs, overall survival was noted to be higher (93% vs. 88%) with surgery and radiation when compared to surgery alone, however this was not statistically significant (P=0.78) (57). This marginal potential benefit, however, has been corroborated in additional studies on intra-cranial SFT suggesting improved responses and outcomes with radiation therapy for patients with incomplete resection or metastatic disease (58,59).…”

Section: Radiation Therapymentioning

confidence: 99%

Solitary fibrous tumor

Davanzo¹,

Emerson²,

Lisy³

et al. 2018

Transl. Gastroenterol. Hepatol

151

171

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Solitary fibrous tumor (SFT) is a rare tumor of mesenchymal origin that account for less than 2% of all soft tissue masses. Initially identified in the pleura, SFT has been identified in multiple anatomic locations and can arise anywhere in the body. The varying histologic features along with non-specific means of identification have led SFT to be associated with several different names. Over the last several decades, sustained advances through research and technology have led to more reliable methods for differentiating this distinct soft tissue tumor. Advances specifically in immunohistochemistry and molecular diagnostics have identified CD34 as the most consistent marker in SFT, however even this lacks specificity to conclusively narrow down the broad differential for exact identification. More recently the discovery of the NAB2-STAT6 fusion gene has led to more precise diagnosis of SFT. Like many other soft tissue tumors, surgical management is the mainstay of treatment for SFT with emphasis on obtaining tumor-negative margins. Radiation therapy and chemotherapy regimens have not demonstrated global effectiveness, and thus no standardized treatments have been identified. Given the rarity of SFT and current supportive evidence for therapies, management should be focused on tumor extirpation. Nonetheless, individualized therapy, determined within a multidisciplinary setting should be considered.

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Section: Radiation Therapymentioning

confidence: 99%

Solitary fibrous tumor

Davanzo¹,

Emerson²,

Lisy³

et al. 2018

Transl. Gastroenterol. Hepatol

151

171

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“…ere is a clear need for more prospective work in this field of oncology, with a diseasespecific focus on SFT. We have seen some interesting multicenter data on the use of pazopanib, axitinib, and radiation therapy in the literature more recently [22,24,25]. Given the epidemiology of SFT, it remains difficult (if not impossible) to perform larger scale, ideally randomized trials to standardize the treatment of this orphan disease, but the sarcoma community should clearly join forces to do more prospective, SFT-specific trials.…”

Section: Sarcomamentioning

confidence: 99%

“…In that series, patients treated with definitive RT (60 Gy) had an objective response rate (ORR) of 67%, achieved local disease control after 5 years in 81.3% of cases, and had a 5 year OS of 87.5%. In the case of palliative RT (39 Gy), the ORR was 38% with a 5-year local control rate of 62.5% and a 5-year OS of 54.2% [22]. Data on the use of pre-or postoperative therapy (RT and/or systemic treatments) are very limited in this sarcoma subtype, though responses observed in advanced disease clearly support the use of RT in selected patients.…”

Section: Introductionmentioning

confidence: 96%

Clinical presentation, natural history and therapeutic approach in patients with solitary fibrous tumor: A retrospective analysis.

Schöffski

Timmermans²,

Hompes

et al. 2019

JCO

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e22522 Background: Solitary fibrous tumor (SFT), a rare variant of soft tissue sarcoma (STS), is characterized by the presence of a NAB2-STAT6 fusion. Given the orphan character of SFT we performed a retrospective analysis. Methods: We retrospectively reviewed all patients (pts) with SFT treated in our institution between 12/1990 - 09/2017. Results: We identified 94 SFT pts (incl. hemangiopericytoma) with a med. follow-up for 4.7 yrs. Common anatomic sites were chest (33%), abdomen (21.3%), brain (12.8%) and extremities (9.6%). The symptomatology at diagnosis was variable. Only 6.4% presented with synchronous metastasis. Hypoglycemia (Doege-Potter syndrome) was seen in 2.1% of cases. A resection of the primary SFT was done in 86 pts (91.5%), their disease-free survival was 35.5 mo and 43% stayed SFT-free during follow-up. Local recurrence occurred in 26.7% of cases, associated with a med. overall survival (OS) of 45.1 mo. Metachronous metastasis was seen in 30.2% of pts, occurring after a med. follow-up of 36 mo. Med. OS after diagnosis of metastasis was 19.0 mo. Systemic therapy was given to 92.9% of pts with inoperable/metastatic disease. The most common 1st line therapy was doxorubicin single agent (57.7% of pts), achieving responses in 13.3% of pts. 2nd line therapies included ifosfamide and pazopanib (31.3% of pts each), 3rd line treatment was very heterogeneous. Conclusions: SFT is an orphan malignancy with a variable clinical course, low incidence of distant spread at first diagnosis but considerable risk of local failure and metachronous metastasis. Surgery is the only curative option at diagnosis, time of relapse and in case of resectable metastasis. Palliative systemic therapy is considered in pts with inoperable/metastatic disease but achieves low response rates. The natural course and survival outcomes of SFT cases treated with palliative intent tend to be better than in non-selected STS pts.

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“…The potential added value of (neo‐)adjuvant RT in combination with surgery, both in the setting of a gross total resection or after less radical surgery for LC and survival, remains unclear 1,5,15‐23 . The outcome for patients after definitive RT, without surgery, has recently been described by our group 24 …”

Section: Introductionmentioning

confidence: 99%

Extrameningeal solitary fibrous tumors—surgery alone or surgery plus perioperative radiotherapy: A retrospective study from the global solitary fibrous tumor initiative in collaboration with the Sarcoma Patients EuroNet

Haas

Walraven

Lecointe-Artzner³

et al. 2020

Cancer

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Background Solitary fibrous tumor (SFT) is a rare mesenchymal malignancy. Although surgery is potentially curative, the local relapse risk is high after marginal resections. Given the lack of prospective clinical trial data, the objective of the current study was to better define the role of perioperative radiotherapy (RT) in various SFT presentations by location. Methods This was retrospective study performed across 7 sarcoma centers. Clinical information was retrieved from all adult patients with extrameningeal, primary, localized SFT who were treated between 1990 and 2018 with surgery alone (S) compared with those who also received perioperative RT (S+RT). Differences in treatment characteristics between subgroups were tested using analysis of variance statistics and propensity score matching. Local control and overall survival rates were calculated from the start of treatment until progression or death from any cause. Results Of all 549 patients, 428 (78%) underwent S, and 121 (22%) underwent S+RT. The median follow‐up was 52 months. After correction for mitotic count and surgical margins, S+RT was significantly associated with a lower risk of local progression (hazard ratio, 0.19: P = .029), an observation further confirmed by propensity score matching (P = .012); however, this association did not translate into an overall survival benefit. Conclusions The results from this retrospective study investigating perioperative RT in patients with primary extrameningeal SFT suggest that combining RT with surgery in the management of this patient population is significantly associated with a reduced risk of local failures, especially in patients who have less favorable resection margins and in those who have tumors with a high mitotic count.

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Radiation Therapy as Sole Management for Solitary Fibrous Tumors (SFT): A Retrospective Study From the Global SFT Initiative in Collaboration With the Sarcoma Patients EuroNet

Cited by 45 publications

References 45 publications

Solitary fibrous tumor

Solitary fibrous tumor

Clinical presentation, natural history and therapeutic approach in patients with solitary fibrous tumor: A retrospective analysis.

Extrameningeal solitary fibrous tumors—surgery alone or surgery plus perioperative radiotherapy: A retrospective study from the global solitary fibrous tumor initiative in collaboration with the Sarcoma Patients EuroNet

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