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Clinical Presentation, Natural History, and Therapeutic Approach in Patients with Solitary Fibrous Tumor: A Retrospective Analysis
Journal Contribution - Journal Article
Background. Solitary fibrous tumor (SFT) is a rare variant of soft tissue sarcoma (STS). Materials and Methods. We reviewed SFTpatients (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%). Medianprogression-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 amFU 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%) withinoperable/metastatic disease. /e most common (57.7%) upfront therapy was doxorubicin, achieving responses in 13.3% of ptswith a PFS of 4.8 m (0.4–23.8). In second line, pts were treated with ifosfamide or pazopanib, the latter achieving the highestresponse rates. /ird-line treatment was heterogeneous. Conclusion. SFT is an orphan malignancy with a highly variable clinicalcourse and a considerable risk of local failure and metachronous metastasis. Surgery is the only curative option; palliative systemictherapy is used in inoperable/metastatic cases but achieves low response rates. /e highest response rates are seen with pazopanibin second/third line.
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Number of pages: 9