Adriana Fonseca1, Samina Afzal2,3, Lynette Bowes4, Bruce Crooks2, Valerie Larouche5, Nada Jabado6, Sebastien Perreault7, Donna L Johnston8, Shayna Zelcer9, Adam Fleming10, Katrin Scheinemann10,11,12, Mariana Silva13, Magimairajan Issai Vanan14, Chris Mpofu15, Beverly Wilson16, David D Eisenstat16, Lucie Lafay-Cousin17, Juliette Hukin18, Cynthia Hawkins19, Ute Bartels19, Eric Bouffet19. 1. Division of Haematology Oncology, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, M5G 1X8, Canada. adriana.fonseca@sickkids.ca. 2. IWK Health Center, Dalhousie University, Halifax, B3K 6R8, Canada. 3. Al Jalila Children's Specialty Hospital, Dubai, United Arab Emirates. 4. Janeway Child Health Center, Memorial University, St. John's, A1B 3V6, Canada. 5. CHU de Québec- Université Laval, Laval University, Québec, G1V 4G2, Canada. 6. Montreal Children's Hospital, McGill University, Montreal, H4A 3J1, Canada. 7. Centre Hospitalier Universitaire Sainte, Justine, Université de Montreal, Montreal, H3T 1C5, Canada. 8. Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, K1H 8L1, Canada. 9. London Health Sciences Children's Hospital, Western University, London, N6A 5A5, Canada. 10. McMaster Children's Hospital, McMaster University, Hamilton, L8P 1H1, Canada. 11. Department of Pediatrics, Kantonsspital Aarau, Aarau, Switzerland. 12. University Children's Hospital Basel, University of Basel, Basel, Switzerland. 13. Kingston General Hospital, Queens University, Kingston, K7L 2V7, Canada. 14. Cancer Care Manitoba, Pediatrics and Child Health, University of Manitoba, Winnipeg, R3E 0V9, Canada. 15. Jim Pattison Children's Hospital, University of Saskatchewan, Saskatoon, S7N 0W8, Canada. 16. Stollery Children's Hospital, University of Alberta, Edmonton, T6G 2B7, Canada. 17. Alberta Children's Hospital, University of Calgary, Calgary, T3B 6A8, Canada. 18. BC Children's Hospital, University of British Columbia, Vancouver, V6H 3N1, Canada. 19. Division of Haematology Oncology, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, M5G 1X8, Canada.
Abstract
BACKGROUND: Diffuse intrinsic pontine gliomas (DIPG) are midline gliomas that arise from the pons and the majority are lethal within a few months after diagnosis. Due to the lack of histological diagnosis the epidemiology of DIPG is not completely understood. The aim of this report is to provide population-based data to characterize the descriptive epidemiology of this condition in Canadian children. PATIENTS AND METHODS: A national retrospective study of children and adolescents diagnosed with DIPG between 2000 and 2010 was undertaken. All cases underwent central review to determine clinical and radiological diagnostic characteristics. Crude incidence figures were calculated using age-adjusted (0-17 year) population data from Statistics Canada. Survival analyses were performed using the Kaplan-Meier method. RESULTS: A total of 163 patients with pontine lesions were identified. Central review determined one-hundred and forty-three patients who met clinical, radiological and/or histological criteria for diagnosis. We estimate an incidence rate of 1.9 DIPG/1,000,000 children/year in the Canadian population over a 10 years period. Median age at diagnosis was 6.8 years and 50.3% of patients were female. Most patients presented with cranial nerve palsies (76%) and ataxia (66%). Despite typical clinical and radiological characteristics, histological confirmation reported three lesions to be low-grade gliomas and three were diagnosed as CNS embryonal tumor not otherwise specified (NOS). CONCLUSIONS: Our study highlights the challenges associated with epidemiology studies on DIPG and the importance of central review for incidence rate estimations. It emphasizes that tissue biopsies are required for accurate histological and molecular diagnosis in patients presenting with pontine lesions and reinforces the limitations of radiological and clinical diagnosis in DIPG. Likewise, it underscores the urgent need to increase the availability and accessibility to clinical trials.
BACKGROUND: Diffuse intrinsic pontine gliomas (DIPG) are midline gliomas that arise from the pons and the majority are lethal within a few months after diagnosis. Due to the lack of histological diagnosis the epidemiology of DIPG is not completely understood. The aim of this report is to provide population-based data to characterize the descriptive epidemiology of this condition in Canadian children. PATIENTS AND METHODS: A national retrospective study of children and adolescents diagnosed with DIPG between 2000 and 2010 was undertaken. All cases underwent central review to determine clinical and radiological diagnostic characteristics. Crude incidence figures were calculated using age-adjusted (0-17 year) population data from Statistics Canada. Survival analyses were performed using the Kaplan-Meier method. RESULTS: A total of 163 patients with pontine lesions were identified. Central review determined one-hundred and forty-three patients who met clinical, radiological and/or histological criteria for diagnosis. We estimate an incidence rate of 1.9 DIPG/1,000,000 children/year in the Canadian population over a 10 years period. Median age at diagnosis was 6.8 years and 50.3% of patients were female. Most patients presented with cranial nerve palsies (76%) and ataxia (66%). Despite typical clinical and radiological characteristics, histological confirmation reported three lesions to be low-grade gliomas and three were diagnosed as CNS embryonal tumor not otherwise specified (NOS). CONCLUSIONS: Our study highlights the challenges associated with epidemiology studies on DIPG and the importance of central review for incidence rate estimations. It emphasizes that tissue biopsies are required for accurate histological and molecular diagnosis in patients presenting with pontine lesions and reinforces the limitations of radiological and clinical diagnosis in DIPG. Likewise, it underscores the urgent need to increase the availability and accessibility to clinical trials.
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Authors: Tene A Cage; Sonia P Samagh; Sabine Mueller; Theodore Nicolaides; Daphne Haas-Kogan; Michael Prados; Anu Banerjee; Kurtis I Auguste; Nalin Gupta Journal: Childs Nerv Syst Date: 2013-05-11 Impact factor: 1.475
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Authors: Margot A Lazow; Christine Fuller; Mariko DeWire; Adam Lane; Pratiti Bandopadhayay; Ute Bartels; Eric Bouffet; Sylvia Cheng; Kenneth J Cohen; Tabitha M Cooney; Scott L Coven; Hetal Dholaria; Blanca Diez; Kathleen Dorris; Moatasem El-Ayadi; Ayman El-Sheikh; Paul G Fisher; Adriana Fonseca; Mercedes Garcia Lombardi; Robert J Greiner; Stewart Goldman; Nicholas Gottardo; Sridharan Gururangan; Jordan R Hansford; Tim Hassall; Cynthia Hawkins; Lindsay Kilburn; Carl Koschmann; Sarah E Leary; Jie Ma; Jane E Minturn; Michelle Monje-Deisseroth; Roger Packer; Yvan Samson; Eric S Sandler; Gustavo Sevlever; Christopher L Tinkle; Karen Tsui; Lars M Wagner; Mohamed Zaghloul; David S Ziegler; Brooklyn Chaney; Katie Black; Anthony Asher; Rachid Drissi; Maryam Fouladi; Blaise V Jones; James L Leach Journal: Neuro Oncol Date: 2022-05-04 Impact factor: 13.029
Authors: Nirav Patil; Michael E Kelly; Debra Nana Yeboa; Robin A Buerki; Gino Cioffi; Sweta Balaji; Quinn T Ostrom; Carol Kruchko; Jill S Barnholtz-Sloan Journal: Neuro Oncol Date: 2021-06-01 Impact factor: 12.300