Department of Neurosurgery, Sorbonne University, AP-HP, La Pitié-Salpêtrière Hospital, 47-83, boulevard de L'Hôpital, 75651, Paris Cedex 13, France.
Department of Neuropathology, Sorbonne University, AP-HP, La Pitié-Salpêtrière Hospital, Paris, France.
J Neurooncol. 2021 May;152(3):491-499. doi: 10.1007/s11060-021-03717-7. Epub 2021 Feb 23.
Myxopapillary ependymoma (MPE) is the most frequent tumor affecting the medullary conus. The surgical therapeutic management is still debated and only few studies have focused on the postoperative clinical outcome of patients. This study aimed to demonstrate long-term postoperative outcome and to assess the predictive factors of recurrence as well as the clinical evolution of these patients.
From 1984 to 2019, in four French centers, 101 adult patients diagnosed with MPE were retrospectively included.
Median age at surgery was 39 years. Median tumor size was 50 mm and lesions were multifocal in 13% of patients. All patients benefited from surgery and one patient received postoperative radiotherapy. Gross total resection was obtained in 75% of cases. Sixteen percent of patients presented recurrence after a median follow-up of 70 months. Progression free survival at 5 and 10 years were respectively estimated at 83% and 79%. After multivariable analysis, sacral localization, and subtotal resection were shown to be independently associated with tumor recurrence. 85% of the patients had a favorable evolution concerning pain. 12% of the patients presented a postoperative deterioration of sphincter function and 4% of motor function.
Surgery alone is an acceptable option for MPE patients. Patients with sacral location or incomplete resection are at high risk of recurrence and should be carefully monitored.
黏液乳头型室管膜瘤(MPE)是最常见的累及脊髓圆锥的肿瘤。手术治疗策略仍存在争议,仅有少数研究集中于患者的术后临床转归。本研究旨在展示长期的术后转归,并评估肿瘤复发的预测因素以及这些患者的临床演变。
1984 年至 2019 年间,在法国的四个中心,回顾性纳入了 101 例成人 MPE 患者。
手术时的中位年龄为 39 岁。肿瘤的中位大小为 50mm,13%的患者存在多发病灶。所有患者均接受了手术治疗,1 例患者术后接受了放疗。75%的患者获得了大体全切除。在中位随访 70 个月后,16%的患者出现肿瘤复发。5 年和 10 年无进展生存率分别估计为 83%和 79%。多变量分析显示,骶骨定位和次全切除与肿瘤复发独立相关。85%的患者疼痛得到了良好的控制。12%的患者术后出现括约肌功能恶化,4%的患者出现运动功能恶化。
单独手术是 MPE 患者的可接受选择。骶骨定位或不完全切除的患者复发风险较高,应密切监测。