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儿童和成人复发性颅咽管瘤:长期复发率及治疗

Recurrent craniopharyngiomas in children and adults: long-term recurrence rate and management.

作者信息

Šteňo Juraj, Bízik Ivan, Šteňo Andrej, Matejčík Viktor

机构信息

Department of Neurosurgery, Derer's Faculty Hospital, Comenius University, Limbová 5, 811 04, Bratislava, Slovakia,

出版信息

Acta Neurochir (Wien). 2014 Jan;156(1):113-22; discussion 122. doi: 10.1007/s00701-013-1938-z. Epub 2013 Nov 19.

Abstract

BACKGROUND

The significance of the majority of the factors influencing the recurrence rate (RR) of craniopharyngiomas remains unclear, and the management of this significance is controversial. The present study aimed to evaluate the influence of patient age and tumor topography on the RR, the efficacy of radiotherapy, and the safety of surgery for recurrences.

METHODS

The RR was analyzed in 38 children (follow-up, 2-256 months [mean, 147.6]) and 63 adults (follow-up, 2-221 months [mean, 100.2]. The efficacy of 18 sessions of radiotherapy (13 patients) and the outcome of 52 secondary surgeries (37 patients) were evaluated.

RESULTS

The RR reached 39.5% in children and 22.2% in adults (p = 0.053). After radical tumor removal, the RR in children (36.7%) was significantly higher (p = 0.024) than that in adults (14%). In children after radical removal of intraventricular and extraventricular craniopharyngiomas (IECs), the RR was higher (60%; p = 0.071) than in extraventricular (intrasellar and suprasellar; purely suprasellar extraventricular) tumors (25%). Radical removal of 50% of tumors was achieved (73.1% in children; 26.9% in adults; p = 0.002) in 56.7% of the first and 40.9% of further recurrences. There was no early mortality after 52 surgeries; functional worsening (endocrine, 2; obesity, 2; visual, 3) occurred after 7/52 secondary surgeries. Recurrence occurred after 9/18 sessions of radiotherapy.

CONCLUSIONS

The RR was higher in children than in adults and in IECs relative to other topographic groups. Children with IECs represent a risk group. The efficacy of radiotherapy was inconclusive. Early detection of recurrences enabled safe excision with low morbidity.

摘要

背景

大多数影响颅咽管瘤复发率(RR)的因素的意义仍不明确,对此意义的处理存在争议。本研究旨在评估患者年龄和肿瘤位置对复发率的影响、放疗的疗效以及复发后手术的安全性。

方法

分析了38例儿童(随访时间2 - 256个月[平均147.6个月])和63例成人(随访时间2 - 221个月[平均100.2个月])的复发率。评估了18次放疗(13例患者)的疗效和52次二次手术(37例患者)的结果。

结果

儿童的复发率为39.5%,成人为22.2%(p = 0.053)。肿瘤根治性切除后,儿童的复发率(36.7%)显著高于成人(14%)(p = 0.024)。在儿童中,根治性切除脑室内和脑室外颅咽管瘤(IECs)后的复发率(60%;p = 0.071)高于脑室外(鞍内和鞍上;单纯鞍上脑室外)肿瘤(25%)。在首次复发的56.7%和再次复发的40.9%中,实现了50%肿瘤的根治性切除(儿童为73.1%;成人26.9%;p = 0.002)。52次手术后无早期死亡;52次二次手术中有7次出现功能恶化(内分泌2例;肥胖2例;视力3例)。18次放疗中有9次出现复发。

结论

儿童的复发率高于成人,且IECs相对于其他位置组的复发率更高。患有IECs的儿童是一个风险组。放疗的疗效尚无定论。复发的早期检测能够实现安全切除且发病率低。

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