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Pediatric thalamic tumors in the MRI era: a Canadian perspective
Authors:Paul Steinbok  Chittur Viswanathan Gopalakrishnan  Alexander R. Hengel  Aleksander M. Vitali  Ken Poskitt  Cynthia Hawkins  James Drake  Maria Lamberti-Pasculli  Olufemi Ajani  Walter Hader  Vivek Mehta  P. Daniel McNeely  Patrick J. McDonald  Adrianna Ranger  Michael Vassilyadi  Jeff Atkinson  Scott Ryall  David D. Eisenstat  Juliette Hukin
Affiliation:1.Division of Pediatric Neurosurgery, Department of Surgery,University of British Columbia & British Columbia Children’s Hospital,Vancouver,Canada;2.Division of Neurosurgery,Royal University Hospital,Saskatoon,Canada;3.Department of Radiology,University of British Columbia & British Columbia Children’s Hospital,Vancouver,Canada;4.Division of Pathology, The Hospital for Sick Children,University of Toronto,Toronto,Canada;5.Division of Pediatric Neurosurgery, Department of Surgery,University of Toronto,Toronto,Canada;6.Division of Neurosurgery,McMaster Children’s Hospital,Hamilton,Canada;7.Division of Neurosurgery,Alberta Children’s Hospital,Calgary,Canada;8.Division of Neurosurgery,Stollery Children’s Hospital,Edmonton,Canada;9.Division of Neurosurgery,IWK Health Centre,Halifax,Canada;10.Section of Neurosurgery,Winnipeg’s Children’s Hospital,Winnipeg,Canada;11.Division of Neurosurgery, Children’s Hospital,London Health Sciences Center,London,Canada;12.Division of Neurosurgery,University of Ottawa & Children’s Hospital of Eastern Ontario,Ottawa,Canada;13.Division of Paediatric Neurosurgery,McGill University Health Centre,Montreal,Canada;14.The Arthur and Sonia Labatt Brain Tumour Research Centre,the Hospital for Sick Children,Toronto,Canada;15.Division of Hematology, Oncology and Palliative Care, Department of Pediatrics,University of Alberta & Stollery Children’s Hospital,Edmonton,Canada;16.Division of Neurology and Oncology, Department of Pediatrics,University of British Columbia & British Columbia Children’s Hospital,Vancouver,Canada
Abstract:

Background

Thalamic gliomas are rare. The natural history is unpredictable, and the optimal management of these tumors in children is poorly defined. The aim was to identify outcomes, prognostic factors, and response to various modalities of treatment in a relatively large population of pediatric thalamic tumors from many centers within a fairly homogeneous health care system.

Methods

We performed a Canadian multicenter retrospective review of pediatric thalamic tumors presenting during the MRI era (1989–2012). Radiology and pathology were reviewed by central independent reviewers. Paraffin shavings for RNA extraction were taken and tested for fusion events involving KIAA1549:BRAF. Tumors were classified as unilateral or bithalamic based on their origin on imaging. Univariate and multivariate analyses on factors influencing survival were performed.

Results

Seventy-two thalamic tumors were identified from 11 institutions. Females represented 53 % of the study population, and the mean age at presentation was 8.9 years. Sixty-two tumors were unilateral and 10 bithalamic. Unilateral tumors had a greater propensity to grow inferiorly towards the brainstem. These tumors were predominantly low grade in comparison to bithalamic tumors which were high-grade astrocytomas. The 5-year overall survival was 61?±?13 % for unithalamic tumors compared to 37?±?32 % for bithalamic tumors (p?=?0.097). Multivariate analysis indicated tumor grade as the only significant prognostic factor for unithalamic tumors. Six unilateral tumors, all low grade, were BRAF fusion positive.

Conclusion

Unilateral and bilateral thalamic tumors behave differently. Surgical resection is an appropriate treatment option in unilateral tumors, most of which are low grade, but outcome is not related to extent of resection (EOR). Bilateral thalamic tumors have a poorer prognosis, but the occasional patient does remarkably well. The efficacy of chemotherapy and radiotherapy has not been clearly demonstrated. Novel therapeutic approaches are required to improve the prognosis for malignant unilateral thalamic tumors and bilateral thalamic tumors.
Keywords:
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