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A painless neck swelling and von Recklinghausen
Acta Chirurgica Belgica
|April 12, 2014
Summary
Von Recklinghausen's disease can cause unusual neurofibromas. This case highlights a cervical vagus nerve neurofibroma presenting as a neck swelling in a patient with this genetic disorder.
Area of Science:
- Neurology
- Oncology
- Genetics
Background:
- Von Recklinghausen's disease (neurofibromatosis type 1) is an autosomal dominant disorder.
- It is characterized by the development of neurofibromas, which are tumors arising from nerve sheath cells.
- While typically affecting peripheral nerves, unusual localizations can occur.
Observation:
- A 46-year-old woman with known Von Recklinghausen's disease presented with a painless neck swelling.
- Clinical examination revealed multiple nodules on the right neck.
- MRI demonstrated a large, sausage-like mass compressing the right jugular vein.
Findings:
- Surgical exploration identified a neurofibroma attached to the right vagal and recurrent laryngeal nerves.
- Histopathological examination confirmed a plexiform cervical vagus nerve neurofibroma.
- The tumor was resected with clear margins, preserving the cranial nerves.
Implications:
- This case underscores the importance of considering cranial nerve neurofibromas in patients with Von Recklinghausen's disease presenting with neck masses.
- Early diagnosis and surgical intervention are crucial for managing such complications.
- Understanding these rare presentations aids in comprehensive patient care and management strategies.
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