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. 2020 Mar 26:2020:1385978.
doi: 10.1155/2020/1385978. eCollection 2020.

Clinical Presentation, Natural History, and Therapeutic Approach in Patients with Solitary Fibrous Tumor: A Retrospective Analysis

P Schöffski  1   2 , I Timmermans  1 , D Hompes  3 , M Stas  3 , F Sinnaeve  4 , P De Leyn  5 , W Coosemans  5 , D Van Raemdonck  5 , E Hauben  6 , R Sciot  6 , P Clement  1 , O Bechter  1 , B Beuselinck  1 , F J S H Woei-A-Jin  1 , H Dumez  1 , P Nafteux  5 , T Wessels  1
Affiliations

Clinical Presentation, Natural History, and Therapeutic Approach in Patients with Solitary Fibrous Tumor: A Retrospective Analysis

P Schöffski et al. Sarcoma. .

Erratum in

Abstract

Background: Solitary fibrous tumor (SFT) is a rare variant of soft tissue sarcoma (STS). Materials and Methods. We reviewed SFT patients (pts) treated at our institution between 12/1990 and 09/2017.

Results: We identified 94 pts with a median follow-up (mFU) of 4.7 years (range: 0.1-21.53). Primary sites were the chest (33%), abdomen (21.3%), brain (12.8%), and extremities (9.6%); 6.4% of pts presented with synchronous metastasis. Median overall survival (mOS) from the first diagnosis was 56.0 months (m) (0.3-258.3). Doege-Potter syndrome was seen in 2.1% of pts. Primary resection was performed in 86 pts (91.5%). Median progression-free survival was 34.1 m (1.0-157.1), and 43% of pts stayed SFT-free during FU. Local recurrence occurred in 26.7% after a mFU of 35.5 m (1.0-153.8), associated with an OS of 45.1 m (4.7-118.2). Metachronous metastasis occurred in 30.2% after a mFU of 36.0 m (0.1-157.1). OS in metastatic pts was 19.0 m (0.3-149.0). Systemic therapy was given to 26 pts (27.7%) with inoperable/metastatic disease. The most common (57.7%) upfront therapy was doxorubicin, achieving responses in 13.3% of pts with a PFS of 4.8 m (0.4-23.8). In second line, pts were treated with ifosfamide or pazopanib, the latter achieving the highest response rates. Third-line treatment was heterogeneous.

Conclusion: SFT is an orphan malignancy with a highly variable clinical course and a considerable risk of local failure and metachronous metastasis. Surgery is the only curative option; palliative systemic therapy is used in inoperable/metastatic cases but achieves low response rates. The highest response rates are seen with pazopanib in second/third line.

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Conflict of interest statement

The authors declare that there are no conflicts of interest regarding the publication of this paper.

Figures

Figure 1
Figure 1
Anatomical localization of the primary solitary fibrous tumor.
Figure 2
Figure 2
Kaplan–Meier estimate for the following: (a) mOS. (b) Local PFS after primary surgery. (c) Distant PFS after primary surgery. (d) mOS since diagnosis of metastatic disease. (e) mOS since the start of palliative systemic therapy. Survival curves (Kaplan–Meier estimates).
Figure 3
Figure 3
Overview of clinical course of patients included in our retrospective analysis. n number of patients, M+: metastasis.
Figure 4
Figure 4
Overview of systemic treatments used in solitary fibrous tumor. (a) First-line systemic treatment (n = 26). (b) Second-line systemic treatment (n = 16). (c) Third-line systemic treatment (n = 10).
See this image and copyright information in PMC

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