Multiple primary intramedullary ependymomas: a case report and review of the literature

Abstract

Background context: Intramedullary ependymomas constitute the most frequent type of intramedullary tumor. In patients with neurofibromatosis type 2 (NF2), multiple intramedullary ependymomas are known to occur. In the non-NF2 population, however, the presence of multiple synchronous intramedullary ependymomas is exceedingly rare.

Purpose: In this article, the authors report the second case in the literature of multiple primary synchronous intramedullary ependymomas. To the best of the authors knowledge, this report represents the first to provide a detailed pathology of all lesions, thereby giving an added level of confidence on the primary synchronous nature of the lesions. The authors have also performed a review of the literature regarding multifocal intramedullary ependymomas.

Study design: A review article and case report.

Conclusions: The concomitant localization of two primary intramedullary spinal cord ependymomas in the setting of nongenetic predisposition is an uncommon phenomenon. In this article, the authors present the second report of multiple, synchronous intramedullary ependymomas. A detailed review of the literature reveals that the presence of multiple intramedullary lesions in non-NF2 patients is both rare and deserving of further study.

Keywords: Ependymoma; Intramedullary; Multifocal; Spine; Tumor.