Primary melanocytic schwannoma of cervical sympathetic chain

Background. Primary melanocytic schwannoma arising from the cervical sympat hetic chain is a rare pigmented nerve sheath tumor. Two cases are presented from an academic medical center. Patients and Methods. Patients were initially seen with an enlarging neck m ass associated with sympathetic nervous system dysfunction. Radiography dem onstrated a mass located posterior to the carotid sheath. Primary therapy c onsisted of surgical excision and postoperative radiation therapy. Results. The tumors were found to be melanocytic schwannomas arising from t he cervical sympathetic chain. The pathologic characteristics of this neopl asm are reviewed. One patient remained disease free for 12 years after trea tment, whereas 1 patient died as a result of local recurrence and distant m etastases. Conclusions. Melanocytic schwannoma of the cervical sympathetic chain is a rare nerve sheath tumor of the head and neck that may be misdiagnosed as ma lignant melanoma. The clinical behavior of this neoplasm is variable. Preop erative neurologic findings, anatomic location, electron microscopy, and im munohistochemistry findings help to establish the diagnosis, and electron m icroscopy may have a role in distinguishing between benign and malignant le sions. Complete surgical excision is the treatment of choice. (C) 2000 John Wiley & Sons, Inc.