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Clinical outcomes and natural history of pediatric brainstem tumors: with 33 cases follow-ups

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Abstract

The purpose of this study is to investigate the patient characteristics and clinical outcomes of children with pediatric brainstem glioma. Between 2004 and 2009, a total of 42 children were diagnosed with brainstem gliomas at the Neurosurgical Center of Beijing Tiantan Hospital, China. A retrospective study including the 33 patients of this cohort with complete follow-up was conducted in an attempt to better understand clinical outcomes following multidisciplinary treatment modalities for pediatric brainstem gliomas. Investigational variables including clinical presentations, anatomical distribution, radiological findings, and clinical outcomes were analyzed. Survival time difference was computed using a Kaplan–Meier method with a log-rank test between groups. The Cox proportional hazards regression model was utilized in the multivariate analysis to determine the independent prognostic factors. Overall median survival of the entire series of patients was 11 months with a 1-year actuarial survival rate of 43.6 %. In nine patients who received no treatment after diagnosis, all patients expired within 8 months with a median time of 3.5 months. On univariate analysis, the following variables including older age at diagnosis, higher Karnofsky Performance Status score at diagnosis, the lower pathological grade, surgical resection modality, increasing diagnostic latency, and focal growth pattern were associated with better survival. On multivariate analysis, only the last two variables were associated with survival advantage. Focal pediatric brainstem gliomas amenable to a surgical resection are likely to achieve a prolonged survival. Clinical trials on larger number of patients are of importance in further understanding this spectrum of devastating diseases.

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Acknowledgments

The study is supported by the National Natural Science Foundation of China for Weiqing Wan (no. 81172192).

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Authors and Affiliations

  1. Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, 6 Tiantan Xili Chongwen District, Beijing, 100050, People’s Republic of China

    Tao Sun, Weiqing Wan, Zhen Wu, Junting Zhang & Liwei Zhang

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Correspondence to Liwei Zhang.

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Comments

Oliver Bozinov, Zurich, Switzerland

The article “Clinical outcomes and natural history of pediatric brainstem tumors: with 33 cases follow-ups” by Tao Sun et al. is a series of brainstem gliomas in a pediatric population with and without treatment. The study was performed in a retrospective way, and conclusions were drawn based on their single-center results. This is a 5-year series with 42 pediatric patients, but only 33 children were included because of missing medical records or failed histological proof. Regarding this special illness, such a series is still considered large. However, the histology and especially the natural course of these patients remain very heterogeneous. The presented Kaplan–Meier curves illustrate very nicely such a course of progression and survival. I still do not agree completely how the conclusion is drawn, that prolonged survival is achieved by surgery. The authors have not proved this scientifically as both groups are very heterogeneous. I agree that surgery in selected (benign) cases should be considered and most likely prolongs life, but the authors could not prove this in a homogenous group statistically. Most likely, such a difficult answer can only be achieved in a multicenter prospective study, which will be a very difficult task. My conclusion based on the presented data is that focal gliomas lead to more surgical interventions and seem to have a prolonged survival outcome in an interdisciplinary approach; thus, careful surgery remains a therapy of choice in selected cases of pediatric brainstem gliomas.

James T. Rutka, Toronto, Canada

The authors have reviewed their experience over 5 years with 42 children with brainstem tumours. The overall median survival of this series was 11 months. Children who had a focal brainstem tumour did better in terms of survival after surgery. It is critical these days to categorize paediatric brainstem tumours into their recognized subgroups primarily based on location by MRI. These categories include: focal midbrain (tectal or tegmental) gliomas, dorsally exophytic pontine, focal pontine, and diffuse intrinsic pontine gliomas; and cervicomedullary brainstem gliomas. Surgery is rarely offered for tectal gliomas, except for CSF diversionary procedures to overcome hydrocephalus, and for diffuse intrinsic pontine gliomas, unless there are some atypical features about these tumours which mandate a biopsy. The gliomas in other locations of the brainstem frequently require neurosurgical approaches and resection. Sadly, the diffuse intrinsic pontine glioma, which is the most aggressive and fatal tumour, is the most common of all paediatric brainstem gliomas.

The authors performed surgery for the most part on the focal brainstem tumours which is a suggested treatment option. The use of advanced microneurosurgical techniques, intraoperative neuromonitoring, and neuronavigation can provide the required safety to ensure that patients will not suffer undue consequences neurologically from surgery. For patients with focal tegmental midbrain gliomas which are pilocytic astrocytomas, surgery alone can be curative, and the authors have shown us such a case.

The future of paediatric brainstem gliomas will be to develop an increased understanding of the molecular genetics of the diffuse gliomas, as these are the lesions that require our utmost attention and help. Fortunately, a number of clinical trials are evolving throughout the world where procurement of tissue will be mandated, and sophisticated high-resolution genotyping of these tumours will be performed. This will then lead us to improved therapies for these tumours with rather dismal prognosis at this time.

Dr. Sun and Dr. Wan contributed equally to this paper.

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Sun, T., Wan, W., Wu, Z. et al. Clinical outcomes and natural history of pediatric brainstem tumors: with 33 cases follow-ups. Neurosurg Rev 36, 311–320 (2013). https://doi.org/10.1007/s10143-012-0428-8

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