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Clival Chordomas: Contemporary Results
Director of the Anterior Skull Base Surgery Center and a Member of the Lateral Skull Base Surgery Program at NYU Langone, New York, USA
Director of the Anterior Skull Base Surgery Center and a member of the Lateral Skull Base Surgery Program at NYU Langone, New York
Source of Support: None, Conflict of Interest: None
The present report describes the authors' unique experience in treating a large number of pediatric skull base chordoma patients. Since the advent of modern skull base surgery, clival chordomas remain a challenging disease. Many contemporary surgical series have been published by many skull base surgical teams, but a series composed of pediatric and adolescent chordomas is rare. Chordomas consist of less than 1% of intracranial tumors and has an incidence of 0.8 per 1 million population. Chordomas in the pediatric age group account for about 5% of chordomas, thus underscoring the rarity of the disease. Generally, chordomas in children have been considered more aggressive and have poorer outcomes. However, a recent report from Al Mefty, et al. reported much better outcomes with aggressive skull base surgery aimed at getting a gross total excision (GTR), followed by proton beam radiation. They treated 18 patients and were able to achieve GTR in 55%. Progression free survival (PFS) at 10 years in the GTR group was 89%. Half their patients had chondroid chordomas and the other half had conventional chordomas and no difference in the PFS or OS was reported with either pathology. We had reported a GTR rate of 58% in a group of 65 chordomas in the adult. GTR was the most important factor for progression free survival. GTR was inversely related to the tumor size and anatomical extensions. Wang, et al. reported their experience in treating 238 patients with chordomas at a mean age of 38 years. GTR and near total excision was achieved in 66% of patients. Factors that adversely affected getting GTR were tumor size and recurrent tumors. PFS at 5 years in primary tumors was 51%.
In the present series the authors have reported on 62 patients over a 9 year period, constituting 12% of their total chordoma patients during that period. Gross total and near total excision were achieved in 60% of the cases. Considering that most institutions now use endonasal endoscopic approaches as the primary approach, the present series has used open craniotomy approaches in 56 cases while 17 cases had expanded endonasal endoscopic approach and 5 had transoral approach. The worst chances of getting a GTR was in large tumors, tumors with hard consistency and those located at the craniovertebral junction. Most of their patients had large tumors at presentation. Progression free survival was 46% at five years. Surprisingly they did not find a statistical difference in the PFS between the GTR and non GTR groups although there was a trend towards a better PFS in the former group. 94% of their patients had conventional chordomas. Thus, it appears from this report that chordomas in the pediatric and adolescent age group behave similar to the adult clival chordomas, both in pathology and clinical course. This is an important report on a rarely reported group of patients with clival chordomas. I congratulate the authors for this valuable contribution to the literature on clival chordomas.
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Bai J, Zhang S, Zhai Y, Wang S, Li M, Li C, et al
. A Series of 62 Skull Base Chordomas in Pediatric and Adolescent Patients: Clinical Characteristics, Treatments, and Outcomes. Neurol India 2020;68: [Ahead of Print].
Rassi MS, Maher Hulou M, Almefty K, Bi WL, Pravdenkova S, Dunn IF,et al
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