Endocrine Abstracts

Introduction: Chordomas are rare slowly growing locally aggressive neoplasms of the bone arising from embryonic remnants of notochord. These tumours typically occur in the axial skeleton and have a proclivity for the spheno-occipital region of the skull base. Parasellar/clivus chordomas account for one third of all chordomas.

Methods: We conducted a retrospective chart review of 4 patients with chordomas of the clivus treated in Beaumont Hospital between 2011 and 2017.

Results: Three of the four patients were male. The median age at presentation was 39 years and the median length of follow-up was 36.5 months. All patients presented with headaches, diplopia and cranial nerve palsies. 6th cranial nerve involvement was noted in all cases at the time of the presentation. Magnetic resonance imaging was employed to localise the tumours. 3 of the patients had evidence of local invasion. None of the patients had clinical evidence of tumour metastasis to distant sites. All patients underwent surgery followed by adjuvant high-dose proton radiotherapy. 3 patients had craniotomies, while one patient had EM guided endoscopic trans-nasal tumour excision. There was no post-operative mortality. However 3 of the 4 patients had evidence of post-operative pituitary dysfunction. Recurrence was noted in one patient. Six further surgical excisions were required prior to radiotherapy (time to first recurrence was 22 months). None of the 4 patients returned to their pre-morbid status, as they all have residual cranial nerve palsies.

Conclusion: Chordomas are aggressive invasive tumours. A multidisciplinary approach in a neurosurgical centre is necessary in the management of these rare tumours. The best results in the treatment of chordomas are reported when using surgery and adjuvant radiotherapy.