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Enigmatic entity in childhood: clival chordoma from a tertiary center’s perspective

  • Clinical Article - Pediatrics
  • Published:
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Abstract

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 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.

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Conflicts of interest

All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participations in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript.

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Authors and Affiliations

  1. Department of Neurosurgery, Hacettepe University School of Medicine, Ankara, Turkey

    Burcak Bilginer, Fırat Narin, Sahin Hanalioglu & Nejat Akalan

  2. Neurosurgery Clinic, Antalya Education and Research Hospital, Antalya, Turkey

    Cezmi Çağrı Türk

  3. Department of Radiology, Hacettepe University School of Medicine, Ankara, Turkey

    Kader Karlı Oguz & Burce Ozgen

  4. Department of Pathology, Hacettepe University School of Medicine, Ankara, Turkey

    Figen Soylemezoglu

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  1. Burcak Bilginer
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  2. Cezmi Çağrı Türk
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Correspondence to Burcak Bilginer.

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Bilginer, B., Türk, C.Ç., Narin, F. et al. Enigmatic entity in childhood: clival chordoma from a tertiary center’s perspective. Acta Neurochir 157, 1587–1593 (2015). https://doi.org/10.1007/s00701-015-2510-9

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  • DOI: https://doi.org/10.1007/s00701-015-2510-9

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