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Clinico-Radiological Outcomes in WNT-Subgroup Medulloblastoma.

Authors :
Mani, Shakthivel
Chatterjee, Abhishek
Dasgupta, Archya
Shirsat, Neelam
Pawar, Akash
Epari, Sridhar
Sahay, Ayushi
Sahu, Arpita
Moiyadi, Aliasgar
Prasad, Maya
Chinnaswamy, Girish
Gupta, Tejpal
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