Satyarthee Guru Dutta, Moscote-Salazar Luis Rafael
Department of Neurosurgery, Neurosciences Centre, AIIMS, New Delhi, India.
Department of Neurosurgery, RED LATINO Latin American Trauma and Intensive Neuro-care Organization, Bogota, Colombia.
J Pediatr Neurosci. 2016 Oct-Dec;11(4):324-327. doi: 10.4103/1817-1745.199479.
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.
室管膜瘤通常易于发生在脑室内表面或脊髓中央管。罕见的是，室管膜瘤可在大脑皮质中发生而不附着于脑室结构。然而，在镰状区域的纯轴外间隙中发生这种类似镰状脑膜瘤的情况极为罕见。在医学文献数据库（Medline）和医学期刊数据库（PubMed）中详细检索原发性镰状室管膜瘤（PEFE）仅得到5例孤立的病例报告。所有病例均发生在17岁以上的患者中；然而，我们的病例是一名9岁女孩。PEFE在男性中更常见，男女比例为3:2，平均年龄为28.8岁（范围17 - 43岁），所有病例术中病变质地均为实性。所有病例均接受了手术切除，随后进行辅助放疗；然而，此外，1例还接受了化疗，平均随访期为9个月，但有1例失访。肿瘤的平均大小为5.25厘米（范围1.8 - 7.2厘米）。作者报告了一例独特的PEFE病例，患者为一名9岁女孩，患有大小为8.6厘米×6厘米×5.4厘米的巨大原发性镰状室管膜瘤，手术治疗成功。据作者所知，目前该病例是西方文献中首例发生在生命第一个十年内的小儿轴外镰状室管膜瘤，在1个半月的时间内迅速发展为巨大肿瘤。本文简要回顾了其简要发病机制、临床特征、治疗方法以及相关文献。
