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. 2024 Feb 7;14(4):358.
doi: 10.3390/diagnostics14040358.

Clinico-Radiological Outcomes in WNT-Subgroup Medulloblastoma

Affiliations

Clinico-Radiological Outcomes in WNT-Subgroup Medulloblastoma

Shakthivel Mani et al. Diagnostics (Basel). .

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.

Keywords: WNT; medulloblastoma; multimodality therapy; relapse; survival.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Kaplan–Meier curves of progression-free survival (A) and overall survival (B) for WNT-pathway medulloblastoma in the study cohort.
Figure 2
Figure 2
Representative images of three patients from the study cohort with local recurrence in the tumour bed (A), synchronous local relapse with spinal deposits (B), and extraneural metastases (C) in the WNT-subgroup medulloblastoma. White arrows highlight the site of relapse in all three cases.

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