Tanycytic ependymoma of the filum terminale associated with multiple endocrine neoplasia type 1: first reported case.

BACKGROUND CONTEXT: Ependymoma associated with multiple endocrine neoplasia type 1 (MEN-1) is an extremely rare clinical entity. To the best of our knowledge, only five cases of ependymoma associated with MEN-1 have been previously described. Furthermore, there has been no case of tanycytic ependymoma of the filum terminale associated with MEN-1.

PURPOSE: The present case report illustrates a 53-year-old man with tanycytic ependymoma of the filum terminale associated with MEN-1. We review the literature on ependymoma with MEN-1 and tanycytic ependymoma of the cauda equina region and also discuss the risk of recurrence.

STUDY

DESIGN

A case report.

METHODS

The patient presented with complaints of nocturnal pain in the lower back, accompanied by numbness around the anus and intermittent claudication for approximately 1 year. Magnetic resonance imaging (MRI) identified an intradural-enhancing, large mass lesion at the level from Th12 to L2 vertebrae, with a cranial cystic lesion.

RESULTS

Open-door laminoplasty of the Th12, L1, and L2 and en bloc tumor resection with thickened filum terminale were performed. Histopathologic examination of the tumor specimens showed tanycytic ependymoma (World Health Organization Classification Grade II). At the time of the 2-year and 8-month follow-up examination, MRI did not show tumor recurrence.

CONCLUSIONS

This is the first reported case of this clinical entity. A careful follow-up of patients with this unusual tumor is strongly recommended.