From the Guidelines
The primary treatment for a solitary fibrous tumor (SFT) is complete surgical resection with negative margins, as it offers the best chance of cure, especially when performed by a surgeon with specific sarcoma expertise 1.
Diagnosis
Diagnosis of SFT typically involves imaging studies such as CT or MRI, followed by a biopsy to confirm the presence of SFT through histological examination and immunohistochemical staining, particularly for markers like CD34, STAT6, and BCL2 1.
Treatment
The aim of resection should be complete resection with negative margins while preserving uninvolved organs, considering the low risk for local recurrence in retroperitoneal SFTs 1.
- For tumors that cannot be completely removed or those that are malignant, additional treatments may include radiation therapy, given the sensitivity of SFT to radiation therapy, or systemic therapy 1.
- The use of pazopanib could be considered as upfront therapy for patients with SFT, especially if available, due to its potential anti-angiogenic effects in sensitive histotypes like SFT 1.
Follow-Up
Long-term follow-up is essential as these tumors can recur even years after initial treatment, particularly those with malignant features or incomplete resection 1.
- The interval between follow-up assessments is not strictly evidence-based but could be shorter initially (3–6 monthly) and annually after 5 years, including clinical evaluation and cross-sectional imaging to detect asymptomatic recurrences early 1.
Considerations
- The decision to use adjuvant therapies such as chemotherapy or radiation should be made on a case-by-case basis, considering the specific characteristics of the tumor and the patient's overall health and preferences 1.
- Participation in clinical trials for new and innovative treatments should be encouraged, especially for rare tumors like SFT, where standard treatments may not be as effective 1.
From the Research
Diagnosis of Solitary Fibrous Tumor (SFT)
- Solitary fibrous tumors are mesenchymal lesions that can arise at various sites, most commonly the pleura 2
- Diagnosis is often incidental, with patients being asymptomatic, but symptoms can occur due to local tumor compression 2, 3
- Radiological methods may not be conclusive, and pathological analysis is often required for diagnosis 2, 3
- Immunohistochemical techniques, particularly the presence of the NAB2-STAT6 fusion detected with a STAT6 antibody, are considered the gold standard for diagnosis 2
Treatment of Solitary Fibrous Tumor (SFT)
- Complete surgical resection is the optimal treatment for both primary and local recurrent disease 2, 4, 3
- In cases of suboptimal resection, large disease burden, or advanced recurrence, a multidisciplinary approach may be preferable 2
- Radiotherapy can provide palliation or shrinkage for inoperable local disease 2, 5
- New risk stratification systems can be used to predict the risk of recurrence or metastasis, considering factors such as mitotic rate, age, tumor size, and presence of necrosis 4
- Long-term follow-up post-resection is recommended due to the potential for unpredictable clinical course and recurrence 2, 4, 3
- Novel combined-modality radiosurgery techniques may be considered for treating recurrent SFT, particularly in cases of grade II SFT post-resection 6