Long-term survival case of malignant glomus tumor mimicking “dumbbell-shaped” neurogenic tumor

We report a very rare case of long-term survival case of malignant glomus tumor (MGT) without widespread metastasis.

Sixty-three-year-old woman who experienced multiple relapses over a 40-year period beginning with a scapular lesion since she was at age 21.

After four local recurrences, the patient underwent wide resection of the scapula at age 36. Thirteen years later, the patient had a neck tumor at the same side with the scapulectomy. The tumor was originated from the C6 spinal nerve and was resected. Twelve years after the surgery, the cervical tumor relapsed with myelopathy at age 61. The tumor infiltrated from the extraspinal canal to the intraspinal canal at the C5/C6 foramen and appeared as a dumbbell-like tumor. Furthermore, the local recurrence occurred 2 years after the operation at age 63. The tumors that were detected at age 36 and age 63 were confirmed to be histologically identical. Those were more than 2 cm in size and arose from a deep location. The basement membrane stained positively for collagen type IV and α-smooth muscle actin on immunohistochemistry. Based on these aspects, the tumor was diagnosed as an MGT.

This is the first case report of MGT which lesions at two time points were confirmed to have common histological features and which confirmed the long-term survival over a 40-year period.