Departments of1Neurological Surgery and.
2Division of Pediatric Neurological Surgery, Children's Hospital of Pittsburgh, Pennsylvania; and.
J Neurosurg Pediatr. 2020 Nov 20;27(2):170-179. doi: 10.3171/2020.6.PEDS19733. Print 2021 Feb 1.
Pediatric skull base chordoma is a rare entity that is traditionally considered to display aggressive behavior with an increased risk of recurrence. There is an absence of literature examining outcomes in the pediatric population in general and using the endoscopic endonasal approach (EEA).
The authors retrospectively reviewed all patients with skull base chordomas presenting by the age of 18 years to the Children's Hospital of Pittsburgh or the University of Pittsburgh Medical Center from 2004 to 2019. Clinical outcomes, the number and location of recurrences, and progression-free survival time were determined.
Twenty patients met the study criteria. The most common presenting complaints were diplopia (n = 7), headache (n = 6), and swallowing difficulty (n = 4). Three cases were incidentally discovered. Twelve patients underwent single-stage EEA alone, 2 patients had two-stage EEA, and 6 patients had combined EEA with open far-lateral or extreme-lateral approaches. Fourteen patients underwent gross-total resection (GTR), and 6 patients had near-total resection. Larger tumors were more likely to require staging or a combined approach (86% vs 7%) and were less likely to receive GTR (33% vs 86%) but had comparable recurrence and mortality rates. Five patients developed CSF leaks requiring reoperation, 2 patients developed a permanent abducens nerve palsy, 1 patient suffered an internal carotid artery injury, 1 patient developed an epidural hematoma, and 1 patient developed a subdural empyema. Four (20%) patients had recurrence during follow-up (mean radiographic follow-up 59 months and mean time to local recurrence 19 months). Two patients with recurrence underwent further resection, and 1 patient elected to stop treatment. Both patients who underwent repeat resection experienced a second recurrence, one of whom elected to stop treatment. Both patients who died had an elevated Ki-67 (p = 0.039), one of whom developed de-differentiated histology. A third patient died of progressive spinal metastases without local recurrence and is one of 2 patients who developed postoperative spinal metastases. Both patients whose tumors became de-differentiated progressed from tumors with an initial Ki-67 of 15 or greater (p = 0.035) and received prior radiotherapy to the bulk tumor (p = 0.03).
The majority of pediatric skull base chordomas, when managed at a specialized center with a goal of GTR, may have a better outcome than traditionally believed. Elevated Ki-67 rates may predict poor outcome and progression to de-differentiation.
小儿颅底脊索瘤是一种罕见的实体瘤，传统上被认为具有较高的复发风险，表现出侵袭性。目前尚无文献研究一般儿科人群的预后，也没有使用内镜经鼻入路（EEA）的研究。
作者回顾性分析了 2004 年至 2019 年期间在匹兹堡儿童医院或匹兹堡大学医学中心就诊的年龄在 18 岁以下的颅底脊索瘤患者的所有病例。确定了临床结果、复发次数和位置，以及无进展生存时间。
20 例患者符合研究标准。最常见的首发症状是复视（n=7）、头痛（n=6）和吞咽困难（n=4）。3 例为偶然发现。12 例患者行一期 EEA 单独治疗，2 例患者行二期 EEA，6 例患者行 EEA 联合开颅远外侧或极外侧入路。14 例患者行大体全切除（GTR），6 例患者行近全切除。较大的肿瘤更可能需要分期或联合入路（86% vs 7%），且更不可能行 GTR（33% vs 86%），但复发率和死亡率相当。5 例患者发生脑脊液漏，需再次手术，2 例患者发生永久性展神经麻痹，1 例患者发生颈内动脉损伤，1 例患者发生硬膜外血肿，1 例患者发生硬膜下积脓。随访期间有 4 例（20%）患者复发（平均影像学随访 59 个月，局部复发平均时间 19 个月）。2 例复发患者行进一步切除，1 例患者选择停止治疗。再次切除的 2 例患者均再次复发，其中 1 例患者选择停止治疗。2 例死亡患者的 Ki-67 均升高（p=0.039），其中 1 例发生去分化组织学。第 3 例患者死于进展性脊柱转移，无局部复发，是 2 例发生术后脊柱转移患者之一。2 例肿瘤发生去分化的患者，其肿瘤的初始 Ki-67 均大于 15（p=0.035），且均接受过肿瘤整块放疗（p=0.03）。
在专门的中心，对大多数小儿颅底脊索瘤患者进行 GTR 治疗，其预后可能优于传统观点。高 Ki-67 率可能预示不良预后和向去分化进展。
