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.

CLINICAL CASE

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.

CONCLUSION

Patients with Von Recklinghausen's disease presenting with painless neck swellings may harbour cranial nerve neurofibromas.