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Clinical Presentation, Natural History, and Therapeutic Approach in Patients with Solitary Fibrous Tumor: A Retrospective Analysis
Joint Authors
Clement, Paul M.
Bechter, Oliver E.
Dumez, Herlinde
Hompes, Daphne
Sinnaeve, Friedl
Schöffski, Patrick
Timmermans, I.
Stas, M.
De Leyn, P.
Coosemans, W.
Van Raemdonck, D.
Hauben, E.
Beuselinck, B.
Woei-A-Jin, F. J. S. H.
Nafteux, P.
Wessels, T.
Sciot, Raf
Source
Issue
Vol. 2020, Issue 2020 (31 Dec. 2020), pp.1-9, 9 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2020-03-27
Country of Publication
Egypt
No. of Pages
9
Main Subjects
Abstract EN
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.
American Psychological Association (APA)
Schöffski, Patrick& Timmermans, I.& Hompes, Daphne& Stas, M.& Sinnaeve, Friedl& De Leyn, P.…[et al.]. 2020. Clinical Presentation, Natural History, and Therapeutic Approach in Patients with Solitary Fibrous Tumor: A Retrospective Analysis. Complexity،Vol. 2020, no. 2020, pp.1-9.
https://search.emarefa.net/detail/BIM-1207262
Modern Language Association (MLA)
Schöffski, Patrick…[et al.]. Clinical Presentation, Natural History, and Therapeutic Approach in Patients with Solitary Fibrous Tumor: A Retrospective Analysis. Complexity No. 2020 (2020), pp.1-9.
https://search.emarefa.net/detail/BIM-1207262
American Medical Association (AMA)
Schöffski, Patrick& Timmermans, I.& Hompes, Daphne& Stas, M.& Sinnaeve, Friedl& De Leyn, P.…[et al.]. Clinical Presentation, Natural History, and Therapeutic Approach in Patients with Solitary Fibrous Tumor: A Retrospective Analysis. Complexity. 2020. Vol. 2020, no. 2020, pp.1-9.
https://search.emarefa.net/detail/BIM-1207262
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1207262