科研之友
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摘要
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.
- 出版日期2014-2
