MalignantSoft tissue
CIC- Rearranged Sarcoma
Synonyms: CIC-DUX4 Sarcoma
Rare high-grade round cell sarcoma
Quick Facts
Behaviour
Malignant
Category
Soft tissue
Grade
High
Synonyms
CIC-DUX4 Sarcoma
Category
Soft tissue
Behaviour
Malignant
Grade
High
Gender
Male
Tissue of Origin
Unknown
Epidemiology
- Rare high-grade round cell sarcoma
- Second most common Ewing-like sarcoma after Ewing sarcoma
- Young adults most commonly affected
Clinical Features
- Rapidly growing soft tissue mass
- Often large and deep-seated
- May present with pain
- Aggressive clinical behaviour
Location
- Deep soft tissues of trunk
- Extremities
- Retroperitoneum
Imaging
- MRI: large heterogeneous soft tissue mass
- Necrosis common
- No bone involvement typically
Pathology
Undifferentiated round cells
Genetics
- CIC-DUX4 fusion (most common)
- CIC-FOXO4 fusion
Treatment
- Chemotherapy (Ewing-like regimens used but less effective)
- Wide surgical resection
- Radiotherapy for local control
Prognosis
- Poor - worse than Ewing sarcoma
- 5-year survival 40%
- High metastatic rate
Key Points
- Rare high-grade round cell sarcoma
- More aggressive than Ewing sarcoma
- Often affects young adults in deep soft tissues
Workup - Blood Tests
- FBC, U&E, LFTs
- LDH
Workup - Local Imaging
- Plain radiograph
- MRI primary site with gadolinium
Workup - Biopsy
Core needle biopsy
Workup - Staging
- CT chest/abdomen/pelvis
- PET-CT
Workup - Other
MDM discussion at a sarcoma centre
Follow-up Summary
Medical disclaimer
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