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中国循证儿科杂志

中国循证儿科杂志 ›› 2023, Vol. 18 ›› Issue (2): 124-128.DOI: 10.3969/j.issn.1673-5501.2023.02.009

• 论著 • 上一篇    下一篇

儿童脉络丛癌12例临床特征及生存分析

李苗1a, 杜淑旭1a,王淑梅1b,龚小军1a,任思其1a,张金1a,张臻1a,孙华颖1a,孙艳玲1a, 邱晓光2a,刘巍2b,李春德2b,武万水1a,孙黎明1a


  

  1. 1首都医科大学附属北京世纪坛医院 北京，100038，a 儿科，b 药剂科；2 首都医科大学附属北京天坛医院 北京，100070，a 放疗科 b 小儿神经外科


  • 收稿日期:2023-03-14 修回日期:2023-03-27 出版日期:2023-04-25 发布日期:2023-05-19
  • 通讯作者: 武万水，孙艳玲

Clinical features and survival analysis of 12 children with choroid plexus carcinoma

LI Miao1a, DU Shuxu1a, WANG Shumei1b, GONG Xiaojun1a, REN Siqi1a, ZHANG Jin1a, ZHANG Zhen1a, SUN Huaying1a, SUN Yanling1a, QIU Xiaoguang2a, LIU Wei2b, LI Chunde2b, WU Wanshui1a, SUN Liming1a   

  1. 1 Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China, a. Department of Pediatrics, b. Department of Pharmacy; 2  Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, China, a.Department of Radiotherapy, b.Department of Pediatric Neurosurgery)

  • Received:2023-03-14 Revised:2023-03-27 Online:2023-04-25 Published:2023-05-19
  • Contact: WU Wanshui,SUN Yanling

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摘要： 背景:儿童脉络丛癌（CPC）临床罕见，国内报道较少。 目的:探讨儿童CPC的临床特征、治疗及结局。 设计:病例系列报告。 方法:回顾性分析首都医科大学附属北京世纪坛医院儿科于2017年1月至2022年10月收治的手术后病理确诊的CPC患儿，随访截至 2022年12月31日。截取患儿性别、诊断年龄、临床表现、治疗和随访情况，生存数据采用KaplanMeier法分析。 主要结局指标:总体生存期（OS）和无进展生存期（PFS）。 结果:12例CPC患儿纳入分析，男4例，女8例；中位诊断年龄29.7（5.8~119.6）个月，起病年龄<3岁8例；肿瘤直径≥5 cm 8例，<5 cm 4例；肿瘤位于幕上9例，幕下3例；肿瘤位于脑室系统6例，脑室外累及脑实质6例；起病时发现播散转移2例，无转移10例。12例均接受肿瘤切除手术，全切8例，近全切4例。术后仅行化疗5例（42%），联合放射治疗及化疗7例（58%）。截至末次随访，8例出现肿瘤复发或进展，其中4例因肿瘤进展后死亡。平均OS（56.7±8.8）个月，1、3、5年OS率分别为（83.3±10.8）%、（66.7±13.6）%和（66.7±13.6）%。平均PFS（24.3±7.2）个月，1、3年PFS率分别为 （41.7±14.2）%和（33.3±13.6）%。KaplanMeier单因素分析发现，肿瘤位于幕下3年OS低于幕上（χ2=8.562, P=0.003）；单纯化疗3年OS低于放化疗联合治疗（χ2=8.488, P=0.004）；不同性别、起病年龄（<3岁与3~18岁）、肿瘤直径（≥5 cm与<5 cm）、切除程度、有无转移、是否放化疗对3年PFS的影响差异均无统计学意义（P均>0.05）。 结论:儿童CPC临床罕见，预后差，肿瘤位于幕下及单纯化疗为影响OS的不良预后因素。

关键词: 脉络丛癌, 儿童, 治疗

Abstract: Background:Choroid plexus carcinoma (CPC) is very rare in children, and there are few reports in China about this special cohort. Objective:To investigate the clinical features, treatment and survival outcome in pediatric CPC patients. Design:Case series report. Methods:Children with CPC confirmed by pathology after tumor resection, who were admitted to pediatric department of Beijing Shijitan Hospital from January 2017 to October 2022, were retrospectively analyzed. The last followup was December 31, 2022. The sex, age at diagnosis, clinical features, treatment and followup were collected. KaplanMeier method was adopted for survival analysis. Main outcome measures:Overall survival (OS) and progressionfree survival (PFS). Results:Among the 12 included patients, four were males and eight were females. The median age at diagnosis was 29.7 (range, 5.8119.6) months, with 8 cases less than 3 years old at the time of diagnosis, tumor diameter ≥5cm in 8 cases, and <5cm in 4 cases. Nine cases were supratentorial, and 3 were infratentorial. The tumor was located in ventricular system in 6 cases, and external involvement of brain parenchyma in 6 cases. Two cases experienced metastases (M+) and 10 patients without metastasis (M0) at the time of diagnosis. All the patients received tumor resection with 8 cases under gross total resection (GTR) and 4 near total resection (NTR). Five (42%) cases received chemotherapy only and 7 patients (58%) were treated with both radiotherapy and chemotherapy after surgery. At the time of last followup, 8 cases experienced tumor recurrence or progression, and 4 cases died. The mean OS were (56.7±8.8) months. The 1, 3, 5year OS were (83.3%±10.8)%、(66.7%±13.6)%, and (66.7%±13.6)%, respectively. The mean PFS time were（24.3±7.2）months. The 1, 3year PFS were (41.7%±14.2)% and (33.3%±13.6)%, respectively. The KaplanMeier univariate analysis showed the 3year OS was poorer in children with infratentorial tumors than those with supratentorial tumors (χ2=8.562, P=0.003). And the 3year OS was also lower in patients who received chemotherapy only than those who received radiotherapy in combination with chemotherapy (χ2=8.488, P =0.004). The difference in 3year PFS was not satistically significant in gender,age at diagnosis(< 3 years old and 318 years old) , tumor diameter (<5 cm and ≥5 cm), GTR and NTR, metastasis or not, chemotherapy only or in combination with radiotherapy (P>0.05). Conclusions:CPC is very rare with poor prognosis in children. Children with infratentorial tumors and who received chemotherapy only tend to have poorer OS.

Key words: Choroid plexus carcinoma, Children, Treatment