Fibroblastic Reticular Cell Tumour
Synonyms: Fibroblastic Reticular Cell Sarcoma
Slow-growing, painless mass
Quick Facts
Behaviour
Malignant
Category
Soft tissue
Grade
Variable
Synonyms
Fibroblastic Reticular Cell Sarcoma
Category
Soft tissue
Behaviour
Malignant
Grade
Variable
Gender
M = F
Tissue of Origin
Fibroblastic Reticular Cell
Epidemiology
- Very rare tumour - only 25-30 described in literature
- Average age 61 - age 13-80
Clinical Features
- Slow-growing, painless mass
- Systemic symptoms (fever, weight loss) are rare unless the disease is advanced
Location
- Most occur in the lymph nodes: cervical and mediastinal nodes most common
- Extranodal site include spleen, soft tissues, liver, and lung
Imaging
Well-defined or infiltrative soft-tissue mass
Pathology
Characterised by spindled to ovoid cells in storiform, whorled, or fascicular patterns.
Genetics
No specific genetic alterations or translocations have been definitively established
Treatment
- Limited evidence
- Primary treatment is wide surgical excision
- Adjuvant radiotherapy or chemotherapy may be considered for high-risk or metastatic case
Prognosis
- Limited data, spontaneous regression to rapid progression has been seen
- Local disease 2-year survival rates around 85.7%
- Metastatic or distant disease at presentation median survival approximately one year
Workup - Blood Tests
Non-specific; may include LDH or FBC to rule out other hematologic conditions.
Workup - Local Imaging
MRI with contrast
Workup - Biopsy
Core needle biopsy at sarcoma centre
Workup - Staging
- CT chest
- PET-CT
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.