caa a22 4500 60621397X CHVBK 20210128101050.0 cr unu---uuuuu 210128e20150901xx s 000 0 eng 10.1007/s00701-015-2510-9 doi (NATIONALLICENCE)springer-10.1007/s00701-015-2510-9 Enigmatic entity in childhood: clival chordoma from a tertiary center's perspective [Elektronische Daten] [Burcak Bilginer, Cezmi Türk, Fırat Narin, Sahin Hanalioglu, Kader Oguz, Burce Ozgen, Figen Soylemezoglu, Nejat Akalan] Purpose: 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 15years in a tertiary care institute. Results: There were two boy and four girls with a mean age of 10.6years (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 (6.9years: 1-14years). 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. Springer-Verlag Wien, 2015 Children nationallicence Chordoma nationallicence Clival nationallicence Endoscopy nationallicence Management nationallicence Radiotherapy nationallicence Bilginer Burcak Department of Neurosurgery, Hacettepe University School of Medicine, Ankara, Turkey aut Türk Cezmi Neurosurgery Clinic, Antalya Education and Research Hospital, Antalya, Turkey aut Narin Fırat Department of Neurosurgery, Hacettepe University School of Medicine, Ankara, Turkey aut Hanalioglu Sahin Department of Neurosurgery, Hacettepe University School of Medicine, Ankara, Turkey aut Oguz Kader Department of Radiology, Hacettepe University School of Medicine, Ankara, Turkey aut Ozgen Burce Department of Radiology, Hacettepe University School of Medicine, Ankara, Turkey aut Soylemezoglu Figen Department of Pathology, Hacettepe University School of Medicine, Ankara, Turkey aut Akalan Nejat Department of Neurosurgery, Hacettepe University School of Medicine, Ankara, Turkey aut Acta Neurochirurgica Springer Vienna 157/9(2015-09-01), 1587-1593 0001-6268 157:9<1587 2015 157 701 https://doi.org/10.1007/s00701-015-2510-9 text/html Onlinezugriff via DOI BK010053 XK010053 XK010000 Metadata rights reserved Springer special CC-BY-NC licence nationallicence 1 research-article jats NATIONALLICENCE NATIONALLICENCE NL-springer NATIONALLICENCE 856 40 https://doi.org/10.1007/s00701-015-2510-9 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Bilginer Burcak Department of Neurosurgery, Hacettepe University School of Medicine, Ankara, Turkey aut NATIONALLICENCE 700 1- Türk Cezmi Neurosurgery Clinic, Antalya Education and Research Hospital, Antalya, Turkey aut NATIONALLICENCE 700 1- Narin Fırat Department of Neurosurgery, Hacettepe University School of Medicine, Ankara, Turkey aut NATIONALLICENCE 700 1- Hanalioglu Sahin Department of Neurosurgery, Hacettepe University School of Medicine, Ankara, Turkey aut NATIONALLICENCE 700 1- Oguz Kader Department of Radiology, Hacettepe University School of Medicine, Ankara, Turkey aut NATIONALLICENCE 700 1- Ozgen Burce Department of Radiology, Hacettepe University School of Medicine, Ankara, Turkey aut NATIONALLICENCE 700 1- Soylemezoglu Figen Department of Pathology, Hacettepe University School of Medicine, Ankara, Turkey aut NATIONALLICENCE 700 1- Akalan Nejat Department of Neurosurgery, Hacettepe University School of Medicine, Ankara, Turkey aut NATIONALLICENCE 773 0- Acta Neurochirurgica Springer Vienna 157/9(2015-09-01), 1587-1593 0001-6268 157:9<1587 2015 157 701