Clinicopathological Characteristics of non-WNT/non-SHH Medulloblastoma Cases in a Pediatric Egyptian Cohort

Abstract

Objective: Medulloblastoma is the most common malignant pediatric brain tumor. It has a great impact on global health. Although, current treatment modalities improve patients` survival rates, survivors suffer from long term treatment related morbidity. Major advances have changed molecular understanding of medulloblastoma with the emergence of the molecular classification of medulloblastoma that has been introduced in WHO classification of CNS tumors. Non-WNT/non-SHH molecular subgroup is the most common molecular subgroup representing more than 60% of medulloblastoma cases. The present study aims to describe the clinical, pathological, and survival characteristics of pediatric patients with non-WNT/non-SHH MB. Results: A total of 36 non-WNT/non-SHH MB cases were detected. The age of the patients ranged between 2 to 18 years. 14 patients (39%) were standard risk while 22 patients (61%) were high risk. Microscopic evaluation showed that 34 cases (94.4%) were of classic histology, while 2 cases (5.6%) were of LC/A histology. The 5-year overall survival of the 36 non-WNT/non-SHH cases detected was 55% and the 5-year event free survival was 40%. Conclusion: The clinical, pathological, and molecular characteristics of pediatric patients with non-WNT/non-SHH molecular subgroup of medulloblastoma described in the present study were mostly similar to those reported in the literature.