MalignantSoft tissue
Adult Fibrosarcoma
Synonyms: Fibrosarcoma of soft tissue
Extremely rare malignancy
Quick Facts
Behaviour
Malignant
Category
Soft tissue
Grade
Variable
Synonyms
Fibrosarcoma of soft tissue
Category
Soft tissue
Behaviour
Malignant
Grade
Variable
Gender
M = F
Tissue of Origin
Fibrous
Epidemiology
- Rare, <1% diagnosis of exclusion - other sarcoma types must be excluded first
- Peak incidence in adults 40–70 years
- Accounts for <5% of soft tissue sarcomas
Clinical Features
- Deep soft tissue mass, often painless
- Slow to rapidly growing depending on grade
- May be large at presentation
Location
- Extremities (thigh most common)
- Trunk
- Head and neck
Imaging
- MRI: non-specific deep soft tissue mass
- Heterogeneous signal with possible necrosis
- No calcification
Pathology
- Herringbone pattern of monomorphic spindle cells
- Variable mitotic rate and necrosis by grade
- Diagnosis of exclusion: no specific differentiation
Genetics
- No defining genetic alterations have been identified
- Diagnosis requires exclusion of other spindle cell sarcomas
Treatment
- Wide local excision with clear margins
- Adjuvant radiotherapy for high-grade or close margins
- Chemotherapy for high-grade disease
Prognosis
- 5-year survival 50–60% for high-grade disease
- Metastasis to lungs, bone and lymph nodes
Key Points
- Extremely rare malignancy
- Diagnosis of exclusion - must rule out other sarcoma variants
- Spindle shaped fibroblasts in herringbone formation
- Grade determines prognosis and treatment
- Radiologic-pathologic correlation is essential
Workup - Blood Tests
FBC, U&E, LFTs - baseline
Workup - Local Imaging
MRI with contrast of primary site
Workup - Biopsy
- Core needle biopsy at sarcoma centre
- Extensive IHC panel to exclude other diagnoses
Workup - Staging
- CT chest/abdomen/pelvis
- PET-CT if high-grade
Workup - Other
MDT review with sarcoma specialist centre
Follow-up Summary
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.