Department of Neurosurgery, Oslo University Hospital, Oslo, Norway.
Department of Neurosurgery, Faculty of Medicine, University of Oslo, Oslo, Norway.
Br J Neurosurg. 2024 Apr;38(2):514-522. doi: 10.1080/02688697.2021.1914821. Epub 2021 Jun 7.
Long-term outcome after surgical treatment of supratentorial ependymoma (STE) in children has not been extensively reported.
We identified 26 children who underwent primary tumor resection of STE between 1953 and 2011, with at least 8 years follow-up. Ten patients (38%) had anaplastic and 16 had low grade ependymoma. Four of 15 children (26%) treated in the years 1953-1976 survived more than 5 years, but the observed 10-year survival was only 7%. One patient lived for 37 years, and second surgery for a local recurrent lesion disclosed a glioblastoma, possibly secondary to radiotherapy. In contrast, the observed 5-year survival rate for 11 children treated in the years 1992-2011 was 8/11 (73%) and observed 10- and 25-year survival rates were 70% and 66%, respectively. Eight patients were alive and tumor-free with follow-up periods of 8-27 (median 18) years, all treated after 1992. Five of these long-term survivors were 23-39 years old with full-time (n = 3) or part-time (n = 2) work. The last three patients were still children (9-12 years old): one with good function and two with major neurological deficits. The majority of patients (n = 18) received adjuvant radiotherapy and eight children no adjuvant treatment. Repeated resections for residual or recurrent tumor were necessary in 11 patients (42%), mostly due to local disease with progressive clinical symptoms. Eight patients underwent only one repeat resection, whereas three patients had two or more repeat resections within 18 years after initial surgery. Four patients were tumor-free after repeated resections at the latest follow-up, 2-13 years after last surgery.
Pediatric STE has a marked risk for local recurrence even after gross total resection and postoperative radiotherapy, but survival has increased following the introduction of modern treatment in recent years. Repeated surgery is an important part of treatment and may lead to persistent tumor control.
儿童幕上室管膜瘤（STE）的手术治疗后长期预后尚未得到广泛报道。
我们确定了 26 名在 1953 年至 2011 年间接受 STE 原发肿瘤切除术的患儿，这些患儿的随访时间至少为 8 年。10 名患者（38%）为间变性，16 名患者为低级别室管膜瘤。1953-1976 年治疗的 15 名儿童中有 4 名（26%）存活时间超过 5 年，但观察到的 10 年生存率仅为 7%。一名患者存活了 37 年，第二次手术切除局部复发病灶显示为胶质母细胞瘤，可能与放疗有关。相比之下，1992-2011 年治疗的 11 名儿童的 5 年生存率为 8/11（73%），观察到的 10 年和 25 年生存率分别为 70%和 66%。8 名患者无肿瘤存活，随访时间为 8-27 年（中位 18 年），均在 1992 年后接受治疗。这 8 名长期生存者中有 5 名年龄为 23-39 岁，全职（n=3）或兼职（n=2）工作。最后 3 名患者仍为儿童（9-12 岁）：1 名功能良好，2 名有严重神经功能缺损。大多数患者（n=18）接受了辅助放疗，8 名儿童未接受辅助治疗。11 名患者（42%）因残留或复发性肿瘤需要反复切除，主要是由于局部疾病伴有进行性临床症状。8 名患者仅进行了一次重复切除，而 3 名患者在初次手术后 18 年内进行了两次或更多次重复切除。4 名患者在最后一次随访时无肿瘤，最后一次手术在 2-13 年前。
即使在大体全切除和术后放疗后，儿童 STE 仍有明显的局部复发风险，但近年来采用现代治疗方法后，生存率有所提高。重复手术是治疗的重要组成部分，可能导致持续的肿瘤控制。
