Dermal Nerve Sheath Tumour
Synonyms: Pacinian neurofibroma (outdated)
Benign neural tumour of dermis
Quick Facts
Behaviour
Benign
Category
Soft tissue
Synonyms
Pacinian neurofibroma (outdated)
Category
Soft tissue
Behaviour
Benign
Gender
M = F
Tissue of Origin
Neural
Epidemiology
- Rare benign neural tumour of dermis
- Adults most commonly affected - 3rd and 4th decade most common
Clinical Features
- Small dermal or subcutaneous nodule
- Slow growing
Location
- Fingers and hand
- Face
- Knee
Imaging
MRI: small dermal nodule, ppears as a well-circumscribed, T2-hyperintense (bright) nodule, consistent with its high myxoid content
Pathology
- Lobulated myxoid nodules in dermis
- No atypia
- S100 positive, SOX10 positive
Genetics
SporadicNo specific recurrent translocations. It is not typically associated with Neurofibromatosis
Treatment
Simple excision curative
Prognosis
- Excellent - recurrence after complete excision is uncommon
- Higher local recurrent rate if incompletely excised
- No malignant potential
Key Points
- Benign neural tumour of dermis
- Lobulated myxoid nodules
- Simple excision curative
Workup - Blood Tests
No blood tests required
Workup - Local Imaging
Clinical diagnosis usually
Workup - Biopsy
Excisional biopsy diagnostic
Workup - Staging
Not required
Follow-up Summary
- 1
Benign but high-recurrence lesion, it follows a "low-risk" surveillance pathway.
- 2
Frequency
Clinical review at 6 months and 12 months post-excision. MRI is only used if recurrence is suspected in a deep or sensitive area.
- 3
Discharge after 1–2 years of stability, with instructions to return if a new lump appears.
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