Chinese Journal of Evidence-Based Pediatrics ›› 2022, Vol. 17 ›› Issue (6): 443-447.DOI: 10.3969/j.issn.1673-5501.2022.06.007

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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

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