Malignant nerve sheath tumor of the jugular foramen followed by meningeal dissemination in a patient with neurofibromatosis.

A 20-year-old female with von Recklinghausen's disease became aware of hoarseness and dysphagia with a right neck mass, and received subtotal resection of a jugular foramen neurofibroma. One year later the jugular foramen tumor recurred and pathologic examination at excision showed a malignant transformation. Despite postoperative chemotherapy and radiotherapy, it recurred soon and followed the metastatic cerebral tumor by meningeal dissemination. Malignant nerve sheath tumors of the jugular foramen are very rare and the clinical course and pathological pictures of this tumor are reviewed.