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Clinico-Radiological Outcomes in WNT-Subgroup Medulloblastoma.
- Source :
-
Diagnostics (2075-4418) . Feb2024, Vol. 14 Issue 4, p358. 15p. - Publication Year :
- 2024
-
Abstract
- Medulloblastoma (MB) comprises four broad molecular subgroups, namely wingless (WNT), sonic hedgehog (SHH), Group 3, and Group 4, respectively, with subgroup-specific developmental origins, unique genetic profiles, distinct clinico-demographic characteristics, and diverse clinical outcomes. This is a retrospective audit of clinical outcomes in molecularly confirmed WNT-MB patients treated with maximal safe resection followed by postoperative standard-of-care risk-stratified adjuvant radio(chemo)therapy at a tertiary-care comprehensive cancer centre. Of the 74 WNT-MB patients registered in a neuro-oncology unit between 2004 to 2020, 7 patients accrued on a prospective clinical trial of treatment deintensification were excluded, leaving 67 patients that constitute the present study cohort. The median age at presentation was 12 years, with a male preponderance (2:1). The survival analysis was restricted to 61 patients and excluded 6 patients (1 postoperative mortality plus 5 without adequate details of treatment or outcomes). At a median follow-up of 72 months, Kaplan–Meier estimates of 5-year progression-free survival and overall survival were 87.7% and 91.2%, respectively. Traditional high-risk features, large residual tumour (≥1.5 cm2), and leptomeningeal metastases (M+) did not significantly impact upon survival in this molecularly characterized WNT-MB cohort treated with risk-stratified contemporary multimodality therapy. The lack of a prognostic impact of conventional high-risk features suggests the need for refined risk stratification and potential deintensification of therapy. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 20754418
- Volume :
- 14
- Issue :
- 4
- Database :
- Academic Search Index
- Journal :
- Diagnostics (2075-4418)
- Publication Type :
- Academic Journal
- Accession number :
- 175653893
- Full Text :
- https://doi.org/10.3390/diagnostics14040358