Solitary Fibrous Tumor.pptx
- 2. Solitary fibrous tumors (SFTs) • Solitary fibrous tumors (SFTs), also known as localized fibrous tumors or fibrous mesotheliomas, are rare spindle cell neoplasms first reported as arising from the pleura. • Solitary fibrous tumors from all anatomic sites usually follow a benign clinical course; however, SFTs can recur and metastasize after surgical resection. • with equal frequency in men and women. Gold, J.S., Antonescu, C.R., Hajdu, C., Ferrone, C.R., Hussain, M., Lewis, J.J., Brennan, M.F. and Coit, D.G. (2002), Clinicopathologic correlates of solitary fibrous tumors. Cancer, 94: 1057-1068. https://doi.org/10.1002/cncr.10328
- 3. • incidence of about 0.2 in 100,000 per year • seen in patients of all ages but predominantly in the fifth and sixth decades of life • usually present as slowly growing masses causing symptoms owing to local compression and/or metastatic disease that can also affect the bone 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
- 4. Histologically, SFTs can be subdivided into • Typical - mitotic count < 4), • Malignant - mitotic count >5, and • Dedifferentiated variants • SFT is consistently associated with NAB2-STAT6 gene fusions arising from recurrent intrachromosomal rearrangements on chromosome 12q 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
- 5. • Lesions tend to present as slowly growing painless masses or may generate symptoms due to mass or pressure effects on adjacent structures. • Uncommonly, FT can present with symptoms of hypoglycemia due to secretion of insulin-like growth factors. • The etiology of SFT remains unknown, and no association has been demonstrated with environmental factors such as tobacco consumption or asbestos exposure.
- 6. Radiology • these are often large, well-defined, lobulated, solid, and vascular masses, often with prominent feeding vessels or sometimes a visible fatty component, which displace adjacent structures. • Radiologic features correlating better with malignancy have included tumor size, heterogeneous signal intensity, and heterogeneous contrast uptake on magnetic resonance imaging
- 8. • While the majority of SFTs with classical morphologic features behave in an indolent manner, the behavior of SFT is unpredictable. • SFTs that have overtly malignant histologic features tend to be aggressive neoplasms that behave as high-grade sarcomas. • Dedifferentiation in SFT is associated with a worse prognosis
- 9. TREATMENT • standard treatment of primary localized SFTs is wide surgical resection. • estimated recurrence rates for malignant SFTs are around 30%.
- 10. Radiotherapy • surgery plus postoperative RT revealed a significant benefit favoring RT in local control. • no significant benefit was seen for OS. • SFT cases at the limit of resectability, or those cases in which a marginal resection is foreseen, especially those cases with a high mitotic rate, could benefit from neoadjuvant RT
- 11. The Role of Postoperative Radiotherapy in Intracranial Solitary Fibrous Tumor/Hemangiopericytoma: A Multi- institutional Retrospective Study (KROG 18-11) • A total of 133 patients with histologically confirmed HPC were included from eight institutions. • Gross total resection (GTR) and subtotal resection (STR) were performed in 86 and 47 patients, respectively. PORT was performed in 85 patients (64%) • The 10-year PFS, and OS rates were 45%, and 71%, respectively. The multivariate analysis suggested that PORT significantly improved LC (p < 0.001) and PFS (p < 0.001). • The PFS benefit of PORT was maintained in the subgroup of GTR (p=0.001), WHO grade II (p=0.001), or STR (p < 0.001). In the favorable subgroup of GTR and WHO grade II, PORT was also significantly related to better PFS (p=0.028). WHO grade III was significantly associated with poor DMFS (p=0.029). • In the PORT subgroup, the 0–0.5 cm margin of the target volume showed an inferior LC to a large margin with 1.0–2.0 cm (p=0.021). Time-dependent Cox proportion analysis showed that distant failures were significantly associated with poor OS (p=0.003).
- 12. 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 • 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.
- 13. Chemotherapy • Chemotherapy has typically been used in the advanced or metastatic setting of SFT patients. However, very limited prospective evidence on the activity of standard cytotoxic drugs is available in SFT
- 15. • While the prognosis is usually excellent for most SFT without malignant histologic features, in view of the unpredictable nature of this disease, long-term follow-up is mandatory. • SFT with malignant features are predictive of poorer outcomes with overall median survival ranging from 59 to 94 months. • Tumor size (>10 cm) and high mitotic rates have been reported as predictive of a higher incidence of metastatic disease. • Common metastatic sites include the lungs, bones, and liver.
- 16. Thank you
Editor's Notes
- #9: it is crucial that patients with SFT are followed up on a long-term basis.