Enigmatic entity in childhood: XXXX from a tertiary center's perspective.

@entity1 @entity129 @entity5 @entity27 @entity3580 @entity892 @entity10
PURPOSE: @entity3580 is a rare @entity5 that arises from embryonic notochordal remnants along the axial skeleton (i.e., clivus, sacrum) and the vertebral bodies. They comprise less than 1 % of CNS @entity5 and 1-4 % of all bone @entity5 . It rarely affects @entity1 and adolescents (<5 %). @entity3580 are locally aggressive and highly recurrent. Their management is challenging for clinicians. METHODS: This retrospective study includes six pediatric @entity1 with pathological evidence of @entity3580 . These cases were identified over a period of 15 years in a tertiary care institute. RESULTS: There were two @entity1 and four @entity1 with a mean age of 10.6 years (range, 4-16 years). The chief complaint was due to @entity129 (or dysfunction), mostly affecting lower @entity129 (66.6 %), followed by @entity892 and @entity10 . One @entity1 had @entity27 . All @entity1 were operated and a total of 15 surgeries were performed (mean, 2.5). @entity5 recurrence was observed in four @entity1 (67 %). Two-year and 5-year progression-free survivals (PFS) were 67 and 33 %, respectively. None of the @entity1 were lost either during the surgery or the follow-up period (6.9 years: 1-14 years). CONCLUSIONS: Clival @entity3580 are challenging @entity5 in neurosurgical practice. A multidisciplinary approach is warranted in each @entity1 . Today, the best management strategy seems to be surgical resection followed by radiotherapy. Chemotherapy should be considered in selective and preference basis. Sharing institutional experiences will provide future insights in prognosis of these rare @entity5 . Implementing newer surgical instruments, endoscope in particular, is encouraged in management of the clival @entity3580 .
