Pediatric Clival Chordoma: A Curable Disease that Conforms to Collins' Law

Neurosurgery
Marcio S RassiOssama Al-Mefty

Abstract

Skull base chordomas in children are extremely rare. Their course, management, and outcome have not been defined. To describe the preeminent clinical and radiological features in a series of pediatric patients with skull base chordomas and analyze the outcome of a cohort who underwent uniform treatment. We emphasize predictors of overall survival and progression-free survival, which aligns with Collins' law for embryonal tumors. Thirty-one patients with a mean age of 10.7 yr (range 0.8-22) harboring skull base chordomas were evaluated. We retrospectively analyzed the outcomes and prognostic factors for 18 patients treated by the senior author, with uniform management of surgery with the aim of gross total resection and adjuvant proton-beam radiotherapy. Mean follow-up was 119.2 mo (range 8-263). Abducens nerve palsy was the most common presenting symptom. Imaging disclosed large tumors that often involve multiple anatomical compartments. Patients undergoing gross total resection had significantly increased progression-free survival (P = .02) and overall survival (P = .05) compared with those having subtotal resection. Those who lived through the period of risk for recurrence without disease progression had a higher probability ...Continue Reading

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Citations

Aug 1, 2020·Frontiers in Neurology·Samantha E HoffmanWenya Linda Bi
Aug 10, 2017·Acta neurochirurgica·Ossama Al-Mefty
Dec 1, 2020·International Journal of Radiation Oncology, Biology, Physics·Daniel J IndelicatoJulie A Bradley
Aug 17, 2019·International Journal of Radiation Oncology, Biology, Physics·Jérôme DoyenBeate Timmermann
Jun 23, 2021·Neurosurgical Review·Gui-Jun ZhangHuan Li
Jul 16, 2019·Clinical Oncology : a Journal of the Royal College of Radiologists·M M HulouJ L Villano
Oct 15, 2021·Acta neurologica Belgica·Taylor ReardonBrian Fiani

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