Paul Steinbok1, Chittur Viswanathan Gopalakrishnan2, Alexander R Hengel2, Aleksander M Vitali3, Ken Poskitt4, Cynthia Hawkins5, James Drake6, Maria Lamberti-Pasculli6, Olufemi Ajani7, Walter Hader8, Vivek Mehta9, P Daniel McNeely10, Patrick J McDonald11, Adrianna Ranger12, Michael Vassilyadi13, Jeff Atkinson14, Scott Ryall15, David D Eisenstat16, Juliette Hukin17. 1. Division of Pediatric Neurosurgery, Department of Surgery, University of British Columbia & British Columbia Children's Hospital, 4480 Oak St., Room K3-159, V6H 3V4, Vancouver, BC, Canada. psteinbok@cw.bc.ca. 2. Division of Pediatric Neurosurgery, Department of Surgery, University of British Columbia & British Columbia Children's Hospital, 4480 Oak St., Room K3-159, V6H 3V4, Vancouver, BC, Canada. 3. Division of Neurosurgery, Royal University Hospital, Saskatoon, SK, Canada. 4. Department of Radiology, University of British Columbia & British Columbia Children's Hospital, Vancouver, BC, Canada. 5. Division of Pathology, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada. 6. Division of Pediatric Neurosurgery, Department of Surgery, University of Toronto, Toronto, ON, Canada. 7. Division of Neurosurgery, McMaster Children's Hospital, Hamilton, ON, Canada. 8. Division of Neurosurgery, Alberta Children's Hospital, Calgary, AB, Canada. 9. Division of Neurosurgery, Stollery Children's Hospital, Edmonton, AB, Canada. 10. Division of Neurosurgery, IWK Health Centre, Halifax, NS, Canada. 11. Section of Neurosurgery, Winnipeg's Children's Hospital, Winnipeg, MB, Canada. 12. Division of Neurosurgery, Children's Hospital, London Health Sciences Center, London, ON, Canada. 13. Division of Neurosurgery, University of Ottawa & Children's Hospital of Eastern Ontario, Ottawa, ON, Canada. 14. Division of Paediatric Neurosurgery, McGill University Health Centre, Montreal, QC, Canada. 15. The Arthur and Sonia Labatt Brain Tumour Research Centre, the Hospital for Sick Children, Toronto, ON, Canada. 16. Division of Hematology, Oncology and Palliative Care, Department of Pediatrics, University of Alberta & Stollery Children's Hospital, Edmonton, AB, Canada. 17. Division of Neurology and Oncology, Department of Pediatrics, University of British Columbia & British Columbia Children's Hospital, Vancouver, BC, 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.
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.
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