Primary melanocytic schwannoma of cervical sympathetic chain.

BACKGROUND

Primary melanocytic schwannoma arising from the cervical sympathetic 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 mass associated with sympathetic nervous system dysfunction. Radiography demonstrated a mass located posterior to the carotid sheath. Primary therapy consisted of surgical excision and postoperative radiation therapy.

RESULTS

The tumors were found to be melanocytic schwannomas arising from the cervical sympathetic chain. The pathologic characteristics of this neoplasm are reviewed. One patient remained disease free for 12 years after treatment, whereas 1 patient died as a result of local recurrence and distant metastases.

CONCLUSIONS

Melanocytic schwannoma of the cervical sympathetic chain is a rare nerve sheath tumor of the head and neck that may be misdiagnosed as malignant melanoma. The clinical behavior of this neoplasm is variable. Preoperative neurologic findings, anatomic location, electron microscopy, and immunohistochemistry findings help to establish the diagnosis, and electron microscopy may have a role in distinguishing between benign and malignant lesions. Complete surgical excision is the treatment of choice.