Extra-axial giant falcine ependymoma with ultra-rapid growth in child: Uncommon entity with literature review

Ependymoma tends to occur commonly along either on ventricular surfaces of the brain or central canal of the spinal cord. Rarely, ependymoma can develop in the cerebral cortex without attachment to the ventricular structures. However, such occurrence in the purely extra-axial compartment in the falcine region mimicking falcine meningioma is exceedingly rare. The detailed search of primary falcine ependymoma (PEFE) in Medline and PubMed yielded only five isolated case reports. All cases occurred in patient older than 17 years of age; however, our case was a 9-year-old girl. PEFE tends to occur more commonly in male with a male to female ratio of 3:2, with the mean age of 28.8 years (range 17-43 years), lesion had intra-operatively had solid consistency in all cases. All cases were subjected to surgical resection followed by adjuvant radiotherapy; however, in addition, one case also received chemotherapy, with an average follow-up period of 9 months, however, missing in one case. The mean size of the tumor was 5.25 cm (range 1.8-7.2 cm). The authors present a unique case of PEFE in a 9-year-old girl with 8.6 cm ×6 cm ×5.4 cm-sized giant primary falcine ependymoma, managed surgically successfully. To the best of the authors' knowledge, the current case is the first case of pediatric extra-axial falcine ependymoma occurring within the first decade of life in the western literature, showing rapid evolution over 1½ month's period into a giant size. Brief pathogenesis, clinical feature, and management along with the pertinent literature are reviewed briefly.