%0 Journal Article
%T Clinical Presentation, Natural History, and Therapeutic Approach in Patients with Solitary Fibrous Tumor: A Retrospective Analysis
%A Bechter
%A O.
%A Beuselinck
%A B.
%A Clement
%A P.
%A Coosemans
%A W.
%A De Leyn
%A P.
%A Dumez
%A H.
%A Hauben
%A E.
%A Hompes
%A D.
%A Nafteux
%A P.
%A Schˋffski
%A P.
%A Sciot
%A R.
%A Sinnaeve
%A F.
%A Stas
%A M.
%A Timmermans
%A I.
%A Van Raemdonck
%A D.
%A Wessels
%A T.
%A Woei-A-Jin
%A F. J. S. H.
%J -
%D 2020
%R https://doi.org/10.1155/2020/1385978
%X 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每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每258.3). Doege每Potter syndrome was seen in 2.1% of pts. Primary resection was performed in 86 pts (91.5%). Median progression-free survival was 34.1ˋm (1.0每157.1), and 43% of pts stayed SFT-free during FU. Local recurrence occurred in 26.7% after a mFU of 35.5ˋm (1.0每153.8), associated with an OS of 45.1ˋm (4.7每118.2). Metachronous metastasis occurred in 30.2% after a mFU of 36.0ˋm (0.1每157.1). OS in metastatic pts was 19.0ˋm (0.3每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 4.8ˋm (0.4每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
%U https://www.hindawi.com/journals/sarcoma/2020/1385978/