Benign Triton Tumour
Synonyms: Neuromuscular hamartoma, nerve rhabdomyoma, ectomesenchymal hamartoma
Benign intraneural proliferation composed of mature peripheral nerve and interspersed muscle
Quick Facts
Behaviour
Benign
Category
Soft tissue
Synonyms
- Neuromuscular hamartoma
- nerve rhabdomyoma
- ectomesenchymal hamartoma
Category
Soft tissue
Behaviour
Benign
Gender
M = F
Tissue of Origin
Neural and muscular
Epidemiology
- Extremely rare
- Occurs often in the first decade of life
Clinical Features
May present with neuropathy or plexopathy, muscle atrophy, secondary deformities (cavus deformity, limb growth disturbance)
Location
- Large nerves
- Nerve plexuses
- Cranial nerves
Imaging
MRI: fusiform enlargement of affected nerve, isointense to skeletal muscle in T1 and T2 sequences
Pathology
Smooth or striated muscle fibers among Schwannian nerve fibers
Genetics
Point mutations in CTNNB1
Treatment
- Standard treatment is not clear due to rarity of condition
- Surgical resection for symptomatic cases or tumors with mass effect
- Observation acceptable for for asymptomatic cases
- Intervention may precipitate the development of desmoid type fibromatosis
Prognosis
- Local recurrence in 33%
- 80% develop desmoid type fibromatosis
Key Points
- Benign intraneural proliferation composed of mature peripheral nerve and interspersed muscle
- Pathogenesis unclear, no aetiologic associations known
Workup - Blood Tests
No blood tests required
Workup - Local Imaging
MRI with contrast
Follow-up Summary
- 1
Extended follow-up recommended
Medical disclaimer
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