title: Enigmatic entity in childhood: clival chordoma from a tertiary center's perspective.
abstract: OBJECTIVE
  Chordoma is a rare neoplasm 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 tumors and 1-4 % of all bone malignancies. It rarely affects children and adolescents (<5 %). Chordomas are locally aggressive and highly recurrent. Their management is challenging for clinicians.
  
  
  METHODS
  This retrospective study includes six pediatric patients with pathological evidence of clival chordoma. These cases were identified over a period of 15 years in a tertiary care institute.
  
  
  RESULTS
  There were two boy and four girls with a mean age of 10.6 years (range, 4-16 years). The chief complaint was due to cranial nerve palsy (or dysfunction), mostly affecting lower cranial nerves (66.6 %), followed by diplopia and headache. One patient had obstructive sleep apnea. All patients were operated and a total of 15 surgeries were performed (mean, 2.5). Tumor recurrence was observed in four patients (67 %). Two-year and 5-year progression-free survivals (PFS) were 67 and 33 %, respectively. None of the patients were lost either during the surgery or the follow-up period (6.9 years: 1-14 years).
  
  
  CONCLUSIONS
  Clival chordomas are challenging tumors in neurosurgical practice. A multidisciplinary approach is warranted in each patient. 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 tumors. Implementing newer surgical instruments, endoscope in particular, is encouraged in management of the clival chordomas.
authors.affiliation: Department of Neurosurgery, Hacettepe University School of Medicine, Ankara, Turkey, burcak@tr.net.
authors.forename: Burcak
authors.initials: B
authors.lastname: Bilginer
authors.forename: Cezmi Çağrı
authors.initials: CÇ
authors.lastname: Türk
authors.forename: Fırat
authors.initials: F
authors.lastname: Narin
authors.forename: Sahin
authors.initials: S
authors.lastname: Hanalioglu
authors.forename: Kader Karlı
authors.initials: KK
authors.lastname: Oguz
authors.forename: Burce
authors.initials: B
authors.lastname: Ozgen
authors.forename: Figen
authors.initials: F
authors.lastname: Soylemezoglu
authors.forename: Nejat
authors.initials: N
authors.lastname: Akalan
mesh_terms: D000293:Adolescent; D002648:Child; D002817:Chordoma / Q000175:diagnosis*; D003388:Cranial Fossa, Posterior; D005260:Female; D006801:Humans; D008297:Male; D019292:Skull Base Neoplasms / Q000175:diagnosis*; D062606:Tertiary Care Centers