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Treatment response of CNS high-grade neuroepithelial tumors with MN1 alteration
  • +13
  • Lorena Baroni,
  • Carlos Rugilo,
  • Fabiana Lubieniecki,
  • Claudia Sampor,
  • Candela Freytes,
  • Liana Nobre,
  • Jordan Hansford,
  • Vajiranee Malalasekera,
  • Michal Zapotocky,
  • Andrew Dodgshun,
  • Ofelia Cruz Martinez,
  • Stefan Rutkowski,
  • Ulrich Schüller,
  • Eric Bouffet,
  • Vijay Ramaswamy,
  • Daniel Alderete
Lorena Baroni
SickKids
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Carlos Rugilo
Hospital Garrahan
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Fabiana Lubieniecki
Hospital de Pediatría Prof. Dr. Juan P. Garrahan
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Claudia Sampor
Hospital de Pediatría "Prof Dr JPGarrahan"
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Candela Freytes
Hospital de Pediatría "Prof Dr JPGarrahan"
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Liana Nobre
Hospital for Sick Children
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Jordan Hansford
Royal Children's Hospital
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Vajiranee Malalasekera
Children Cancer's Centre
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Michal Zapotocky
Charles University
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Andrew Dodgshun
Christchurch Hospital
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Ofelia Cruz Martinez
Hospital Sant Joan de Deu
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Stefan Rutkowski
University Medical Center Hamburg-Eppendorf
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Ulrich Schüller
Universitatsklinikum Hamburg Eppendorf Institut fur Neuropathologie
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Eric Bouffet
Hospital for Sick Children
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Vijay Ramaswamy
Hospital for Sick Children
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Daniel Alderete
Hospital de Pediatría Prof. Dr. Juan P. Garrahan
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Abstract

CNS high-grade neuroepithelial tumor with MN1 alteration (CNS HGNET-MN1) is a rare entity recently described. Thirteen CNS HGNET-MN1 patients were identified using genome wide methylation arrays/RT-PCR across 7 institutions and were correlated with treatment and outcome. All patients had surgery (GTR:9/STR:4) as initial management followed by radiotherapy (focal:5/craniospinal:2/CyberKnife:1) and systemic chemotherapy in 3 patients. Seven patients relapsed; 5 local and 2 metastatic despite complete resection and radiotherapy. Three patients died due to their tumor relapse after 24 months despite upfront radiotherapy. Treatment of CNS HGNET-MN1 remains a major challenge with multiple failures, despite aggressive surgical resections and upfront radiotherapy.

Peer review status:ACCEPTED

06 May 2020Submitted to Pediatric Blood & Cancer
06 May 2020Submission Checks Completed
06 May 2020Assigned to Editor
21 May 2020Reviewer(s) Assigned
21 Jun 2020Review(s) Completed, Editorial Evaluation Pending
22 Jun 2020Editorial Decision: Revise Minor
14 Jul 20201st Revision Received
14 Jul 2020Submission Checks Completed
14 Jul 2020Assigned to Editor
15 Jul 2020Review(s) Completed, Editorial Evaluation Pending
16 Jul 2020Editorial Decision: Accept