Margot A Lazow1,2,3, Christine Fuller4, Mariko DeWire1, Adam Lane1, Pratiti Bandopadhayay5, Ute Bartels6, Eric Bouffet6, Sylvia Cheng7, Kenneth J Cohen8, Tabitha M Cooney5, Scott L Coven9, Hetal Dholaria10, Blanca Diez11, Kathleen Dorris12, Moatasem El-Ayadi13, Ayman El-Sheikh14, Paul G Fisher15, Adriana Fonseca6, Mercedes Garcia Lombardi16, Robert J Greiner17, Stewart Goldman18, Nicholas Gottardo10, Sridharan Gururangan19, Jordan R Hansford20, Tim Hassall21, Cynthia Hawkins22, Lindsay Kilburn23, Carl Koschmann24, Sarah E Leary25, Jie Ma26, Jane E Minturn27, Michelle Monje-Deisseroth1, Roger Packer23, Yvan Samson28, Eric S Sandler29, Gustavo Sevlever11, Christopher L Tinkle30, Karen Tsui31, Lars M Wagner32, Mohamed Zaghloul13, David S Ziegler33, Brooklyn Chaney1, Katie Black1, Anthony Asher1, Rachid Drissi3,34, Maryam Fouladi2,3, Blaise V Jones35,36, James L Leach35,36. 1. Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. 2. Pediatric Neuro-Oncology Program, Nationwide Children's Hospital, Columbus, Ohio, USA. 3. The Ohio State University College of Medicine, Columbus, Ohio, USA. 4. Department of Pathology, Upstate Medical University, Syracuse, New York, USA. 5. Dana Farber Cancer Institute, Harvard Cancer Center, Boston, Massachusetts, USA. 6. Division of Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada. 7. Division of Pediatric Hematology/Oncology/BMT, British Columbia Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada. 8. The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Baltimore, Maryland, USA. 9. Division of Oncology, Riley Hospital for Children, Indianapolis, Indiana, USA. 10. Department of Oncology, Perth Children's Hospital, Nedlands, Australia. 11. Department of Oncology and Pathology, Fundacion para la lucha de las enfermedades neurologicas de la infancia FLENI, Buenos Aires, Argentina. 12. Center for Cancer and Blood Disorders, Children's Hospital Colorado, Aurora, Colorado, USA. 13. National Cancer Institute, Cairo University and Children's Cancer Hospital Egypt, Cairo, Egypt. 14. Division of Oncology, Dayton Children's Hospital, Dayton, Ohio, USA. 15. Department of Neurology, Stanford University, Stanford, California, USA. 16. Division of Oncology, Hospital de Ninos Ricardo Gutierrez, Buenos Aires, Argentina. 17. Division of Oncology, Penn State Health Children's Hospital, Hershey, Pennsylvania, USA. 18. Department of Pediatrics, Phoenix Children's Hospital, University of Arizona College of Medicine-Phoenix, Phoenix, Arizona, USA. 19. Department of Neurosurgery, University of Florida, Gainsville, Florida, USA. 20. Children's Cancer Centre, Royal Children's Hospital Murdoch Children's Research Institute University of Melbourne, Melbourne, Victoria, Australia. 21. Division of Oncology, Queensland Children's Hospital, South Brisbane, Australia. 22. Division of Pathology, The Hospital for Sick Children, Toronto, Ontario, Canada. 23. Division of Oncology, Children's National Medical Center, Washinton, District of Columbia, USA. 24. Department of Pediatrics, University of Michigan, Ann Arbor, Michigan, USA. 25. Cancer and Blood Disorders Center, Seattle Children's Hospital, Seattle, Washington, USA. 26. Division of Oncology, Xinhua Hospital, Shanghai, China. 27. Division of Oncology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA. 28. Division of Oncology, CHU Saint Justine, Montreal, Quebec, Canada. 29. Division of Oncology, Nemours Children's Health System, Wilmington, Delaware, USA. 30. Division of Radiation Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee, USA. 31. Division of Oncology, Starship Children's Hospital, Auckland, New Zealand. 32. Division of Pediatric Hematology/Oncology, University of Kentucky, Lexington, Kentucky, USA. 33. School of Women's and Children's Health and Children's Cancer Institute, University of New South Wales, Sydney, Australia. 34. Center for Childhood Cancer & Blood Disorders, Nationwide Children's Hospital, Columbus, Ohio, USA. 35. Department of Radiology and Medical Imaging, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. 36. Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA.
Abstract
BACKGROUND: Diffuse intrinsic pontine glioma (DIPG) remains a clinico-radiologic diagnosis without routine tissue acquisition. Reliable imaging distinction between DIPG and other pontine tumors with potentially more favorable prognoses and treatment considerations is essential. METHODS: Cases submitted to the International DIPG registry (IDIPGR) with histopathologic and/or radiologic data were analyzed. Central imaging review was performed on diagnostic brain MRIs (if available) by two neuro-radiologists. Imaging features suggestive of alternative diagnoses included nonpontine origin, <50% pontine involvement, focally exophytic morphology, sharply defined margins, and/or marked diffusion restriction throughout. RESULTS: Among 286 patients with pathology from biopsy and/or autopsy, 23 (8%) had histologic diagnoses inconsistent with DIPG, most commonly nondiffuse low-grade gliomas and embryonal tumors. Among 569 patients with centrally-reviewed diagnostic MRIs, 40 (7%) were classified as non-DIPG, alternative diagnosis suspected. The combined analysis included 151 patients with both histopathology and centrally-reviewed MRI. Of 77 patients with imaging classified as characteristic of DIPG, 76 (99%) had histopathologic diagnoses consistent with DIPG (infiltrating grade II-IV gliomas). Of 57 patients classified as likely DIPG with some unusual imaging features, 55 (96%) had histopathologic diagnoses consistent with DIPG. Of 17 patients with imaging features suggestive of an alternative diagnosis, eight (47%) had histopathologic diagnoses inconsistent with DIPG (remaining patients were excluded due to nonpontine tumor origin). Association between central neuro-imaging review impression and histopathology was significant (p < 0.001), and central neuro-imaging impression was prognostic of overall survival. CONCLUSIONS: The accuracy and important role of central neuro-imaging review in confirming the diagnosis of DIPG is demonstrated.
BACKGROUND: Diffuse intrinsic pontine glioma (DIPG) remains a clinico-radiologic diagnosis without routine tissue acquisition. Reliable imaging distinction between DIPG and other pontine tumors with potentially more favorable prognoses and treatment considerations is essential. METHODS: Cases submitted to the International DIPG registry (IDIPGR) with histopathologic and/or radiologic data were analyzed. Central imaging review was performed on diagnostic brain MRIs (if available) by two neuro-radiologists. Imaging features suggestive of alternative diagnoses included nonpontine origin, <50% pontine involvement, focally exophytic morphology, sharply defined margins, and/or marked diffusion restriction throughout. RESULTS: Among 286 patients with pathology from biopsy and/or autopsy, 23 (8%) had histologic diagnoses inconsistent with DIPG, most commonly nondiffuse low-grade gliomas and embryonal tumors. Among 569 patients with centrally-reviewed diagnostic MRIs, 40 (7%) were classified as non-DIPG, alternative diagnosis suspected. The combined analysis included 151 patients with both histopathology and centrally-reviewed MRI. Of 77 patients with imaging classified as characteristic of DIPG, 76 (99%) had histopathologic diagnoses consistent with DIPG (infiltrating grade II-IV gliomas). Of 57 patients classified as likely DIPG with some unusual imaging features, 55 (96%) had histopathologic diagnoses consistent with DIPG. Of 17 patients with imaging features suggestive of an alternative diagnosis, eight (47%) had histopathologic diagnoses inconsistent with DIPG (remaining patients were excluded due to nonpontine tumor origin). Association between central neuro-imaging review impression and histopathology was significant (p < 0.001), and central neuro-imaging impression was prognostic of overall survival. CONCLUSIONS: The accuracy and important role of central neuro-imaging review in confirming the diagnosis of DIPG is demonstrated.
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