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Preprint Article Version 1 Preserved in Portico This version is not peer-reviewed

Clinico-radiological outcomes in WNT-subgroup medulloblastoma

Version 1 : Received: 15 January 2024 / Approved: 16 January 2024 / Online: 16 January 2024 (08:35:46 CET)

A peer-reviewed article of this Preprint also exists.

Mani, S.; Chatterjee, A.; Dasgupta, A.; Shirsat, N.; Pawar, A.; Epari, S.; Sahay, A.; Sahu, A.; Moiyadi, A.; Prasad, M.; Chinnaswamy, G.; Gupta, T. Clinico-Radiological Outcomes in WNT-Subgroup Medulloblastoma. Diagnostics 2024, 14, 358. Mani, S.; Chatterjee, A.; Dasgupta, A.; Shirsat, N.; Pawar, A.; Epari, S.; Sahay, A.; Sahu, A.; Moiyadi, A.; Prasad, M.; Chinnaswamy, G.; Gupta, T. Clinico-Radiological Outcomes in WNT-Subgroup Medulloblastoma. Diagnostics 2024, 14, 358.

Abstract

Background: Medulloblastoma (MB) comprises four broad molecular subgroups - 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. WNT-MB has the best outcomes (5-year survival >90%) with contemporary multi-modality treatment prompting attempts at treatment de-intensification to reduce late toxicity. This is a retrospective audit of clinical outcomes in WNT-MB patients treated at a tertiary-care comprehensive cancer centre. Methods: Patients with molecularly confirmed WNT-MB treated with maximal safe resection followed by post-operative standard-of-care risk-stratified adjuvant radio(chemo)therapy were identified retrospectively via electronic search of the neuro-oncology database. Data regarding clinico-demographic characteristics, histo-molecular features, treatment details, patterns of failure, and survival outcomes was retrieved from electronic medical records and/or hospital case files. Time-to-event outcomes were analyzed using Kaplan-Meier methods and compared with the log-rank test. Results: Between 2004 to 2020, 74 patients with WNT-MB were registered in the neuro-oncology unit of the institute. Seven patients treated on a prospective clinical trial of therapy de-intensification were excluded leaving 67 patients that constitute the present study cohort. Median age at presentation was 12 years (inter-quartile range 9-18 years) with a male preponderance (2:1). Six patients (1 post-operative mortality and 5 without adequate details of treatment or outcomes) were excluded from the survival analysis which was restricted to 61 patients. 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 such as large residual tumor (≥1.5cm2) and leptomeningeal metastases (M+) did not significantly impact upon survival in this molecularly-characterized WNT-MB cohort. Conclusions: This retrospective clinical audit confirms excellent survival in WNT-MB patients treated with contemporary multi-modality therapy. Lack of prognostic impact of conventional high-risk features suggests the need for refined risk-stratification and potential de-intensification of therapy.

Keywords

medulloblastoma; multi-modality therapy; relapse; survival; WNT

Subject

Medicine and Pharmacology, Oncology and Oncogenics

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