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Predisposition of Wingless Subgroup Medulloblastoma for Primary Tumor Hemorrhage.

Authors
  • Reisinger, Dominik1
  • Gojo, Johannes1
  • Kasprian, Gregor2
  • Haberler, Christine3
  • Peyrl, Andreas1
  • Azizi, Amedeo A1
  • Mayr, Lisa1
  • Chocholous, Monika1
  • Kool, Marcel4, 5, 6
  • Czech, Thomas7
  • Slavc, Irene1
  • 1 Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria. , (Austria)
  • 2 Department of Radiology, Medical University of Vienna, Vienna, Austria. , (Austria)
  • 3 Institute of Neurology, Medical University of Vienna, Vienna, Austria. , (Austria)
  • 4 Hopp Children's Cancer Center (KiTZ), Heidelberg, Germany. , (Germany)
  • 5 Division of Pediatric Neuro-Oncology, German Cancer Research Center DKFZ, Heidelberg, Germany. , (Germany)
  • 6 German Cancer Consortium DKTK, Heidelberg, Germany. , (Germany)
  • 7 Department of Neurosurgery, Medical University of Vienna, Vienna, Austria. , (Austria)
Type
Published Article
Journal
Neurosurgery
Publication Date
Apr 01, 2020
Volume
86
Issue
4
Pages
478–484
Identifiers
DOI: 10.1093/neuros/nyz148
PMID: 31065705
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

Primary intratumoral hemorrhage as a presenting sign is rare in children with medulloblastomas but may result in severe complications. Given the distinct properties of molecular medulloblastoma subgroups, the impact on neurosurgical practice has still to be defined. To investigate both clinical and radiological presentation of intratumoral hemorrhage in medulloblastoma patients in the context of molecular subgroups. Data of all consecutive medulloblastoma patients treated at our institution between 1993 and 2018 (n = 104) were retrospectively reviewed in respect of clinical and radiological presentation as well as molecular subgroups. For cases with available tumor tissue (n = 86), subgroups were assigned by either 450 K methylation array or immunohistochemistry and CTNNB1 sequencing. Available imaging at diagnosis (n = 62) was reviewed by an experienced neuroradiologist. Within the entire cohort, 4 patients (4%) presented with massive spontaneous hemorrhage. Although no patient died as a direct consequence of hemorrhage, all suffered from serious sequelae. Moreover, 3 additional patients displayed radiological evidence of significant hemorrhage. Interestingly, all 7 cases belonged to the wingless (WNT) subgroup (n = 13), resulting in intratumoral hemorrhage in 54% (7/13) of pediatric WNT medulloblastomas. In contrast, significant hemorrhage was absent in all other molecular subgroups. Our results suggest that a substantial proportion of pediatric WNT medulloblastomas display significant intratumoral hemorrhage at the time of diagnosis. Consequently, the presence of significant hemorrhage in fourth ventricle childhood tumors is suggestive of WNT medulloblastoma and should lead to a less aggressive attempt for total resection in this prognostically favorable tumor type. © Congress of Neurological Surgeons 2019.

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