Malignant Peripheral Nerve Sheath Tumour
Synonyms: MPNST, Malignant schwannoma
High-grade malignancy
Quick Facts
Behaviour
Malignant
Category
Soft tissue
Grade
High
Synonyms
- MPNST
- Malignant schwannoma
Category
Soft tissue
Behaviour
Malignant
Grade
High
Gender
M = F
Tissue of Origin
Neural
Epidemiology
- Malignant nerve sheath tumour
- Peak incidence 2nd-5th decades
- 25-30% associated with NF1
- Often arises from Benign nerve sheath tumour
- Large and deep lesions at Higher risk
Clinical Features
- Rapid growth
- Pain
- Neurological symptoms (compression)
- Constitutional symptoms if metastatic
Location
- Extremities (most common)
- Trunk
- Head and neck
- Retroperitoneum
Imaging
- MRI: heterogeneous High-grade appearance
- Involvement of nerve sheath and soft tissues
- Hemorrhage and necrosis common
Pathology
- High-grade spindle cell sarcoma
- Myxoid or fascicular pattern
- High mitotic rate
- Necrosis typical
- TP53 mutations, other alterations
Genetics
- NF1 loss or inactivation (25-30%)
- TP53, CDKN2A alterations common
Treatment
- Wide surgical resection: primary
- Chemotherapy (MAID or similar): adjuvant
- Radiotherapy: adjuvant if margins or deep
Prognosis
- 50% 5-year survival
- NF1-associated: worse prognosis
- High metastatic potential
- Pulmonary metastases common
Key Points
- High-grade malignancy
- Screen for NF1
- Multimodal therapy standard
- NF1 association worsens prognosis
Workup - Blood Tests
FBC, U&E, LFTs, LDH
Workup - Local Imaging
- MRI primary site: local staging
- CT chest: pulmonary staging
- Whole-body imaging: consider PET-CT
Workup - Biopsy
Core needle or open: confirm High-grade sarcoma
Workup - Staging
- CT chest: pulmonary metastases
- Consider head/abdomen if High-risk
Workup - Other
- MDT at soft tissue sarcoma centre
- NF1 testing if not previously diagnosed
- Genetic counselling
Follow-up Summary
- 1
Year 1
Post-operative visit within first 6 weeks; 3–4 monthly clinical examination and CXR; image prosthesis at 6 months and 1 year
- 2
Year 2
3–4 monthly clinical examination and CXR; image prosthesis annually
- 3
Years 3–4
6-monthly clinical examination and CXR; image prosthesis annually
- 4
Years 5–10
Annual clinical examination and CXR; image prosthesis annually
- 5
Discharge at 10 years after surgery
Medical disclaimer
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