Clinical characteristics and prognostic factors of pediatric diffuse brain stem glioma_West China Medical Journal

Authors：

CUI Wenyao ¹ , HE Wenbo ² , TENG Haibo ² , JU Yan ² , WU Shuwen ¹ , ZHU Li ¹ , XU Jianguo ² ,  LIU Zhiyong ²

1. Department of Neurosurgery, West China Hospital, Sichuan University / West China School of Nursing, Sichuan University, Chengdu, Sichuan 610041, P. R. China;
2. Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding author：

LIU Zhiyong, Email: doctor_lzy@163.com

Keywords：

Children; Diffuse glioma; Brain stem; Clinical feature; Prognostic factor

DOI：

10.7507/1002-0179.202106213

Video：

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Objective To explore the clinical features and prognostic factors of diffuse brain stem glioma in children. Methods A retrospective analysis was conducted on pediatric diffuse brain stem glioma diagnosed by pathology in West China Hospital of Sichuan University between January 2016 and May 2019. The demographic data, clinical manifestations, MRI findings, pathological results, and treatment were included in the prognosis study.Results A total of 39 cases of pediatric diffuse brain stem glioma confirmed by pathology were enrolled, including 21 males and 18 females aged between 3 and 14 years with an average of (8.1±2.8) years and mostly between 5 and 10 years (29 cases). The mean maximum diameter of gliomas was (4.46±0.81) cm. Among the 39 cases, there were 15 cases complicated with hydrocephalus and 16 cases whose tumors completely surrounded the basilar artery. The median survival time was 6 months. The one-year survival rate was 15.4%, and the two-year survival rate was 5.1%. Univariate analyses showed that the tumor enhancement and completely enclosed basilar artery had significant impact on the prognosis (P<0.05). Multiple Cox regression analysis showed that whether the basilar artery was completely wrapped was related to the prognosis [hazard ratio=4.596, 95% confidence interval (1.839, 11.488), P=0.001]. Conclusions Pediatric diffuse brain stem gliomas are common in children aged 5-10 years with poor prognosis. Whether the tumor completely surrounds the basilar artery is closely related to the short overall survival time.

Citation： CUI Wenyao, HE Wenbo, TENG Haibo, JU Yan, WU Shuwen, ZHU Li, XU Jianguo, LIU Zhiyong. Clinical characteristics and prognostic factors of pediatric diffuse brain stem glioma. West China Medical Journal, 2021, 36(8): 1048-1055. doi: 10.7507/1002-0179.202106213 Copy

1.	Coosney T, Lane A, Bartels U, et al. Contemporary survival endpoints: an International Diffuse Intrinsic Pontine Glioma Registry study. Neuro Oncol, 2017, 19(9): 1279-1280.
2.	Hassan H, Pinches A, Picton SV, et al. Survival rates and prognostic predictors of high grade brain stem gliomas in childhood: a systematic review and meta-analysis. J Neurooncol, 2017, 135(1): 13-20.
3.	Baugh J, Bartels U, Leach J, et al. The international diffuse intrinsic pontine glioma registry: an infrastructure to accelerate collaborative research for an orphan disease. J Neurooncol, 2017, 132(2): 323-331.
4.	任艳明, 肖安琪, 杨翔, 等. 弥漫性脑干胶质瘤研究现状. 华西医学, 2016, 31(4): 775-779.
5.	Louis DN, Perry A, Reifenberger G, et al. The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol, 2016, 131(6): 803-820.
6.	Makepeace L, Scoggins M, Mitrea B, et al. MRI patterns of extrapontine lesion extension in diffuse intrinsic pontine gliomas. AJNR Am J Neuroradiol, 2020, 41(2): 323-330.
7.	Tisnado J, Young R, Peck KK, et al. Conventional and advanced imaging of diffuse intrinsic pontine glioma. J Child Neurol, 2016, 31(12): 1386-1393.
8.	Hoffman LM, Veldhuijzen van Zanten SEM, Colditz N, et al. Clinical, radiologic, pathologic, and molecular characteristics of long-term survivors of diffuse intrinsic pontine glioma (DIPG): a collaborative report from the International and European Society for pediatric oncology DIPG registries. J Clin Oncol, 2018, 36(19): 1963-1972.
9.	Wm R, Maher E, Adel M, et al. Pediatric diffuse intrinsic pontine glioma: where do we stand?. Cancer Metastasis Rev, 2019, 38(4): 759-770.
10.	泮长存, 汤劼, 李德志, 等. 儿童弥漫性内生性脑桥胶质瘤的自然病史及预后因素分析. 中华神经外科杂志, 2015, 31(2): 115-118.
11.	Pfaff E, El DA, Balasubramanian GP, et al. Brainstem biopsy in pediatric diffuse intrinsic pontine glioma in the era of precision medicine: the INFORM study experience. Eur J Cancer, 2019, 114(6): 27-35.
12.	Aziz-Bose R, Monje M. Diffuse intrinsic pontine glioma: molecular landscape and emerging therapeutic targets. Curr Opin Oncol, 2019, 31(6): 522-530.
13.	Mueller S, Jain P, Liang WS, et al. A pilot precision medicine trial for children with diffuse intrinsic pontine glioma-PNOC003: a report from the Pacific Pediatric Neuro-Oncology Consortium. Int J Cancer, 2019, 145(7): 1889-1901.
14.	Cooney TM, Lubanszky E, Prasad R, et al. Diffuse midline glioma: review of epigenetics. J Neurooncol, 2020, 150(1): 27-34.
15.	Leach JL, Roebker J, Schafer A, et al. MR imaging features of diffuse intrinsic pontine glioma and relationship to overall survival: report from the International DIPG Registry. Neuro Oncol, 2020, 22(11): 1647-1657.
16.	Hoster E, Rosenwald A, Berger F, et al. Prognostic value of Ki-67 index, cytology, and growth pattern in Mantle-cell lymphoma: results from randomized trials of the European Mantle Cell Lymphoma Network. J Clin Oncol, 2016, 34(12): 1386-1394.
17.	Menon SS, Guruvayoorappan C, Sakthivel KM, et al. Ki-67 protein as a tumour proliferation marker. Clin Chim Acta, 2019, 491(491): 39-45.
18.	Janjua MB, Ban VS, El AT, et al. Diffuse intrinsic pontine gliomas: diagnostic approach and treatment strategies. J Clin Neurosci, 2020, 72(72): 15-19.

1. Coosney T, Lane A, Bartels U, et al. Contemporary survival endpoints: an International Diffuse Intrinsic Pontine Glioma Registry study. Neuro Oncol, 2017, 19(9): 1279-1280.
2. Hassan H, Pinches A, Picton SV, et al. Survival rates and prognostic predictors of high grade brain stem gliomas in childhood: a systematic review and meta-analysis. J Neurooncol, 2017, 135(1): 13-20.
3. Baugh J, Bartels U, Leach J, et al. The international diffuse intrinsic pontine glioma registry: an infrastructure to accelerate collaborative research for an orphan disease. J Neurooncol, 2017, 132(2): 323-331.
4. 任艳明, 肖安琪, 杨翔, 等. 弥漫性脑干胶质瘤研究现状. 华西医学, 2016, 31(4): 775-779.
5. Louis DN, Perry A, Reifenberger G, et al. The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol, 2016, 131(6): 803-820.
6. Makepeace L, Scoggins M, Mitrea B, et al. MRI patterns of extrapontine lesion extension in diffuse intrinsic pontine gliomas. AJNR Am J Neuroradiol, 2020, 41(2): 323-330.
7. Tisnado J, Young R, Peck KK, et al. Conventional and advanced imaging of diffuse intrinsic pontine glioma. J Child Neurol, 2016, 31(12): 1386-1393.
8. Hoffman LM, Veldhuijzen van Zanten SEM, Colditz N, et al. Clinical, radiologic, pathologic, and molecular characteristics of long-term survivors of diffuse intrinsic pontine glioma (DIPG): a collaborative report from the International and European Society for pediatric oncology DIPG registries. J Clin Oncol, 2018, 36(19): 1963-1972.
9. Wm R, Maher E, Adel M, et al. Pediatric diffuse intrinsic pontine glioma: where do we stand?. Cancer Metastasis Rev, 2019, 38(4): 759-770.
10. 泮长存, 汤劼, 李德志, 等. 儿童弥漫性内生性脑桥胶质瘤的自然病史及预后因素分析. 中华神经外科杂志, 2015, 31(2): 115-118.
11. Pfaff E, El DA, Balasubramanian GP, et al. Brainstem biopsy in pediatric diffuse intrinsic pontine glioma in the era of precision medicine: the INFORM study experience. Eur J Cancer, 2019, 114(6): 27-35.
12. Aziz-Bose R, Monje M. Diffuse intrinsic pontine glioma: molecular landscape and emerging therapeutic targets. Curr Opin Oncol, 2019, 31(6): 522-530.
13. Mueller S, Jain P, Liang WS, et al. A pilot precision medicine trial for children with diffuse intrinsic pontine glioma-PNOC003: a report from the Pacific Pediatric Neuro-Oncology Consortium. Int J Cancer, 2019, 145(7): 1889-1901.
14. Cooney TM, Lubanszky E, Prasad R, et al. Diffuse midline glioma: review of epigenetics. J Neurooncol, 2020, 150(1): 27-34.
15. Leach JL, Roebker J, Schafer A, et al. MR imaging features of diffuse intrinsic pontine glioma and relationship to overall survival: report from the International DIPG Registry. Neuro Oncol, 2020, 22(11): 1647-1657.
16. Hoster E, Rosenwald A, Berger F, et al. Prognostic value of Ki-67 index, cytology, and growth pattern in Mantle-cell lymphoma: results from randomized trials of the European Mantle Cell Lymphoma Network. J Clin Oncol, 2016, 34(12): 1386-1394.
17. Menon SS, Guruvayoorappan C, Sakthivel KM, et al. Ki-67 protein as a tumour proliferation marker. Clin Chim Acta, 2019, 491(491): 39-45.
18. Janjua MB, Ban VS, El AT, et al. Diffuse intrinsic pontine gliomas: diagnostic approach and treatment strategies. J Clin Neurosci, 2020, 72(72): 15-19.

West China Medical Journal

Clinical characteristics and prognostic factors of pediatric diffuse brain stem glioma

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