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儿童和青少年颅咽管瘤患者的长期预后:单中心回顾性经验

Long-term outcome of childhood and adolescent patients with craniopharyngiomas: a single center retrospective experience.

作者信息

Zhang Li-Yuan, Du Han-Ze, Lu Ting-Ting, Song Shuai-Hua, Xu Rong, Jiang Yue, Pan Hui

机构信息

Key Laboratory of Endocrinology of National Health Commission, Department of Endocrinology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.

Department of Endocrinology, Jining First People's Hospital, Jining, Shandong, 272000, China.

出版信息

BMC Cancer. 2024 Dec 19;24(1):1555. doi: 10.1186/s12885-024-13352-w.

Abstract

BACKGROUND

The treatment of craniopharyngiomas (CPs) poses challenges due to their proximity to critical neural structures, the risk of serious complications, and the impairment of quality of life after treatment. However, long-term prognostic data are still scarce. Therefore, the purpose of this retrospective study is to evaluate the long-term outcomes of patients with CPs after treatment.

MATERIAL AND METHOD

Our center retrospectively collected data on 83 children and adolescents who underwent craniopharyngioma surgery between 2001 and 2020. The medical records and radiological examination results of the patient were reviewed.

RESULTS

Outcomes were analysed for 80/83 patients who completed follow-up: 50 males (62.5%) and 30 females (37.5%), the median age at the time of diagnosis 8.4 (5.3-12.2) years. The median follow-up time was 136 (61-280) months. The 5-, 10- and 15-year overall survival (OS) rates were 100%, 98.3%, and 94.6%, respectively. Accordingly, the disease-specific survival (DSS) rates were 100%, 98.3% and 94.6%, respectively. Overall progression-free survival (PFS) rates after 5, 10 and 15 years of follow-up in the entire group were 85.4%, 72.2% and 70.1%, respectively. Multivariate analysis found that surgical resection grade was only associated with PFS outcomes [ HR = 0.031 (95% CI: 0.006, 0.163), P < 0.001], without improving OS or DSS. After undergoing recombinant human growth hormone (rhGH) replacement therapy, the total cholesterol (TC) level decreased by 0.90 mmol/L compared to baseline (P = 0.002), and the low-density lipoprotein cholesterol (LDL-C) level decreased by 0.73 mmol/L compared to baseline (P = 0.010). For liver function, compared with baseline data, aspartate aminotransferase (AST) and alanine aminotransferase (ALT) showed a downward trend, but did not reach a statistically significant level (P > 0.05).

CONCLUSION

Surgical treatment of CPs provides good long-time OS and DSS, even though combined with radiotherapy in only selected cases. Gross total resection (GTR) is individual positive prognostic factor. rhGH replacement could improve CPs lipid profile.

摘要

背景

颅咽管瘤(CPs)的治疗具有挑战性,因为它们靠近关键神经结构,存在严重并发症风险,且治疗后生活质量会受损。然而,长期预后数据仍然匮乏。因此,这项回顾性研究的目的是评估CPs患者治疗后的长期结局。

材料与方法

我们中心回顾性收集了2001年至2020年间接受颅咽管瘤手术的83名儿童和青少年的数据。对患者的病历和影像学检查结果进行了审查。

结果

对80/83名完成随访的患者的结局进行了分析:50名男性(62.5%)和30名女性(37.5%),诊断时的中位年龄为8.4(5.3 - 12.2)岁。中位随访时间为136(61 - 280)个月。5年、10年和15年的总生存率(OS)分别为100%、98.3%和94.6%。相应地,疾病特异性生存率(DSS)分别为100%、98.3%和94.6%。整个组随访5年、10年和15年后的无进展生存率(PFS)分别为85.4%、72.2%和70.1%。多因素分析发现手术切除分级仅与PFS结局相关[HR = 0.031(95%CI:0.006,0.163),P < 0.001],并未改善OS或DSS。接受重组人生长激素(rhGH)替代治疗后,总胆固醇(TC)水平较基线下降了0.90 mmol/L(P = 0.002),低密度脂蛋白胆固醇(LDL-C)水平较基线下降了0.73 mmol/L(P = 0.010)。对于肝功能,与基线数据相比,天冬氨酸转氨酶(AST)和丙氨酸转氨酶(ALT)呈下降趋势,但未达到统计学显著水平(P > 0.05)。

结论

CPs的手术治疗提供了良好的长期OS和DSS,尽管仅在部分病例中联合了放疗。全切除(GTR)是个体的阳性预后因素。rhGH替代可改善CPs患者的血脂情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f54c/11661029/3124209b1060/12885_2024_13352_Fig1_HTML.jpg

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