A painless neck swelling and von Recklinghausen.
BACKGROUND
Von Recklinghausen's disease, an autosomal dominant genetic disorder, may occasionally present with unusual localizations of neurofibromas.


METHODS
A 46-year old woman, known with Von Recklinghausen's disease, reported a swelling and a feeling of pressure and globus sensation. Clinical examination revealed multiple large nodi on the right side of the neck. Laboratory testing including thyroid-stimulating hormone (TSH) was normal. An MRI showed a 1 x 3 x 10 centimeter sausage-like structure compressing the right jugular vein. At surgical exploration a string-like tumour firmly attached to the right vagal and recurrent laryngeal nerve was found. The tumor and both nerves were macroscopically curative resected. Histopathological examination demonstrated a plexiform cervical vagus nerve neurofibroma without malignancy.


CONCLUSIONS
Patients with Von Recklinghausen's disease presenting with painless neck swellings may harbour cranial nerve neurofibromas.
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Yazar
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Streukens
D003390:Cranial Nerve Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007823:Laryngeal Nerves; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009455:Neurofibroma / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D009456:Neurofibromatosis 1 / Q000150:complications*; D014630:Vagus Nerve