Solitary Fibrous Tumour
Synonyms: Hemangiopericytoma (now reclassified as SFT)
Intermediate-grade with Variable behaviour
Quick Facts
Behaviour
Intermediate
Category
Soft tissue
Grade
Variable
Synonyms
Hemangiopericytoma (now reclassified as SFT)
Category
Soft tissue
Behaviour
Intermediate
Grade
Variable
Gender
M = F
Tissue of Origin
Fibrous
Epidemiology
- Intermediate-grade mesenchymal tumour
- Peak incidence 4th-6th decades
- Can occur in various sites
- NAB2-STAT6 fusion characteristic
Clinical Features
- Often asymptomatic incidental finding
- Slow-growing
- Larger lesions may cause mass effect
- Hypoglycaemia if large (paraneoplastic)
Location
- Soft tissues (extremities, trunk)
- Pleura and lung
- Meninges
- Liver
- Orbit
Imaging
- MRI: homogeneous or heterogeneous
- T1 isointense, Variable T2
- Well-demarcated
- Significant Vascularity possible
Pathology
- Bland spindle cells in fascicles or storiform pattern
- Prominent branching haemangiopericytoma-like vasculature
- NAB2-STAT6 fusion confirmatory
- STAT6+ on immunostain
Genetics
NAB2-STAT6 fusion 90%
Treatment
- Wide surgical excision: primary treatment
- Risk assessment important (size, mitotic rate)
- Adjuvant radiotherapy: High-risk lesions
- Chemotherapy/VEGF inhibitors: for unresectable or progressive
Prognosis
- Variable based on risk: Low-risk excellent, High-risk 50-60% 5-year
- Recurrence and metastasis possible especially High-risk
Key Points
- Intermediate-grade with Variable behaviour
- Risk assessment scheme guides treatment
- NAB2-STAT6 fusion diagnostic
Workup - Blood Tests
Glucose: screen for hypoglycaemia
Workup - Local Imaging
- MRI primary site: assess size and extent
- CT chest/abdomen: baseline for High-risk
Workup - Biopsy
STAT6 immunostain: NAB2-STAT6 fusion indicator
Workup - Staging
No standard staging
Medical disclaimer
The content on Sarcopedia is for educational and informational purposes only and does not constitute medical advice. It is not intended to be a substitute for professional medical diagnosis or treatment. Always consult with a qualified physician regarding any health concerns or before starting any new treatment. Reliance on any information provided on this site is solely at your own risk.