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中国循证儿科杂志

中国循证儿科杂志 ›› 2022, Vol. 17 ›› Issue (6): 443-447.DOI: 10.3969/j.issn.1673-5501.2022.06.007

• 论著 • 上一篇    下一篇

儿童松果体母细胞瘤的临床特征及预后相关因素分析

李苗1a,孙艳玲1a,王淑梅1b,杜淑旭1a,龚小军1a,任思其1a,高文超1a,万露露1a,武跃芳1a,邱晓光2a,李春德2b,田永吉2b,刘巍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:2022-10-09 修回日期:2022-12-24 出版日期:2022-12-25 发布日期:2022-12-25
  • 通讯作者: 孙黎明;武万水

Analysis of clinical characteristics and prognostic factors of pineoblastoma in children

LI Miao1a, SUN Yanling1a, WANG Shumei1b, DU Shuxu1a, GONG Xiaojun1a, REN Siqi1a, GAO Wenchao1a, WAN Lulu1a, WU Yuefang1a, QIU Xiaoguang2a, LI Chunde2b, TIAN Yongji2b, LIU Wei2b, 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:2022-10-09 Revised:2022-12-24 Online:2022-12-25 Published:2022-12-25
  • Contact: SUN Liming; WU Wanshui

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摘要： 背景 儿童松果体母细胞瘤（PB）临床发病率低，国内少见报道。 目的 探讨PB患儿的临床特征、治疗结局及预后相关因素。 设计 回顾性队列研究。 方法 纳入2017 年 1月至2021年 12月首都医科大学附属北京世纪坛医院收治、经手术病理确诊的PB患儿。通过门诊或电话随访至2022年 8月31日。截取患儿的性别、诊断年龄、临床表现、影像学表现、手术病理检查结果、治疗和随访情况。 主要结局指标 总体生存期 (OS)和无进展生存期(PFS)。 结果 16例PB患儿纳入分析，男15例，女1例；中位诊断年龄 67.6（20.7，129.4）月， <3岁4例。起病时临床表现以颅高压症状为主，无患儿合并明显内分泌功能障碍。MR表现：颅内原发肿瘤表现为松果体区及三脑室后占位， 1例肿瘤扩散至第四脑室；13例术前伴有幕上脑积水； 5例起病时即发现播散转移（M+），其中脊髓播散3例、脑干及脊髓播散1例、骶尾部椎管内种植转移1例。行肿瘤全切12例，近全切4例；手术入路采用经胼胝体-穹窿间入路15例， Poppen入路1例，无明显围手术期并发症；肿瘤Ki-67指数<30% 3例、~60% 5例、~80% 8例。术后先化疗后放疗9例，先放疗后化疗7例。中位随访时间47.6（36.8，56.9）个月。 9例出现复发/进展，其中因肿瘤进展死亡2例。OS 10.8~102.2个月，平均OS（90.7±7.6）个月，1、3、5年OS率分别为 （93.8±6.1）%、（85.9±9.3）%和（85.9±9.3）%。中位PFS 29.5（17.6，50.0）个月，1、3、5年PFS率分别为（87.5±8.3）%、（41.5±13.8）%和（33.2±13.3）%。男童生存率高于女童（χ2=5.405, P=0.02）；肿瘤Ki-67指数为~60%的PB患儿PFS短于<30%者（χ2 =4.600, P=0.032）。COX回归分析显示，肿瘤Ki-67指数~60%的患儿PFS缩短风险是Ki-67指数<30%患儿的3.2倍（HR=3.211, 95% CI：1.027~10.037，P=0.045）。 结论 儿童PB罕见，预后较差，Ki-67指数是PFS的影响因素。

关键词: 松果体母细胞瘤, 儿童, 治疗

Abstract: Background Pineoblastoma are rare central nervous system tumors in children, and very few reports are available about this special cohort in China. Objective To investigate the clinical features, survival outcome and prognostic factors in pediatric pineoblastoma. Design Retrospective cohort study. Methods Children with pineoblastoma (confirmed by pathology) admitted to pediatric department of Beijing Shijitan Hospital from January 2017 to December 2021, were included for clinical analysis. The last follow-up was August 31, 2022 by clinic visit or telephone. The sex, age at diagnosis, clinical manifestations, imaging findings, surgical and pathological results, treatment and follow-up of the patients were included for analysis. Main outcome measures Overall survival (OS) and progression-free survival (PFS). Results Among 16 patients, 15 were males and 1 was female. The median age of onset was 67.6 (range, 20.7-129.4) months, with 4 cases less than 3 years old at the time of diagnosis. The main onset symptoms were intracranial hypertension, and there was no children with obvious endocrine dysfunction. MR images of the primary intracranial tumors showed space-occupying tumors in the pineal region and behind the third ventricle, and tumor spread to the fourth ventricle in one case. Supratentorial hydrocephalus was found in thirteen cases before operation. Five patients experienced metastases (M+) at the time of diagnosis, with spinal cord spread in 3 cases, brainstem and spinal cord spread in one case, and sacral spinal canal metastasis in one case. All the tumors of the sixteen patients were surgically removed with 12 cases under gross total resection and 4 cases near total resection. The surgeries were undertaken through transcallosal interforniceal approach in 15 cases, and Poppen approach in one case, with no obvious perioperative complications in these patients. Ki-67 index was less than 30% in 3 cases, 30%-60% in 5 cases and 61%-80% in 8 cases. All of the patients were treated with both radiotherapy and chemotherapy after surgery, including 9 cases receiving chemotherapy first followed by radiotherapy, and 7 cases receiving chemotherapy following radiotherapy. The median follow-up time was 47.6(36.8, 56.9)months. At last follow-up, nine patients experienced tumor recurrence or progression, and two of them died. The mean OS was 90.7±7.6 (range: 10.8-102.2) months, The 1, 3, 5-year OS were (93.8±6.1)%, (85.9±9.3)% and (85.9±9.3)%, respectively. The median PFS time was 29.5 (17.6, 50.0) months, and the 1, 3, 5-year PFS were (87.5±8.3)%, (41.5±13.8)% and (33.2%±13.3)%, respectively. Univariate analysis showed that boys had better overall survival than girls (χ2=5.405, P=0.02). The PFS of children with Ki-67 index of 30%-60% was shorter than that of <30% (χ2 =4.600, P=0.032). Multivariate COX regression analysis also showed that the risk of PFS in children with Ki-67 index of 30-60% was 3.2 times higher than that in children with Ki-67 index < 30%(HR=3.211, 95% CI: 1.027-10.037, P=0.045) Conclusion Pineoblastoma in children are rare. Ki-67 index is a prognostic factor affecting PFS.

Key words: Pineoblastoma, Children, Treatment