The authors present a case of intraspinal malignant psammomatous melanotic schwannoma (PMS) not associated with Carney complex and review all reported cases not associated with this syndrome. The focus of this review paper is on the characteristics of the malignant progression of PMS. A 54-year-old man had a history of squamous cell carcinoma of the neck and tonsillar carcinoma. The patient's serial CT scanning study showed a mass in the left C-5 foramen. On presentation he was neurologically intact. After 18 months, the patient developed radiating pain down the left arm with decreased sensation. MRI of the cervical spine showed an enhancing 2.1 × 1.5 × 1.9-cm mass in the left C5-6 foramen. A C5-6 hemilaminectomy was performed with gross-total removal of the tumor. At 3 months postoperatively, the patient developed new-onset pain and weakness. MRI showed a dumbbell-shaped mass in the left C-7 foramen. MRI of the pelvis showed a 1.4 × 1.0-cm lesion on the right ischium and a 1.1 × 2.8-cm lesion on the right inferior pubic ramus. Anterior cervical discectomy of C5-6 and C6-7 with corpectomy of C-6 with subtotal resection of the tumor was completed. PMS should not be considered a benign tumor because in 41.1% of patients, including the patient in this report, the tumor progresses to malignancy. Long-term follow-up is needed in these patients. New surgical treatment plans should be considered.
Keywords: Carney complex; MRA = MR angiography; PMS = psammomatous melanotic schwannoma; intraspinal; oncology; psammomatous melanotic schwannoma.