Multimodal MRI Features of Medulloblastoma with Extensive Nodularity and Activated Sonic Hedgehog: A Case Report

Abstract

: Medulloblastoma with extensive nodularity (MBEN) is a rare histological subtype of medulloblastomas in very young children. It is characterized by an aggressive grape-like appearance and favorable outcomes. Herein, we report the case of a nine-month-old patient with MBEN, associated with an activated sonic hedgehog (SHH), with rare multimodal magnetic resonance imaging (MRI) features. Conventional MRI findings indicated the dense homogeneous enhancement of a large unusual gyriform mass in the cerebellar vermis and both cerebellar hemispheres. The mass showed restricted diffusion on diffusion-weighted images, low concentrations of taurine on MR spectroscopy, and a relatively low cerebral blood volume on dynamic susceptibility contrast (DSC) MR perfusion image. The patient underwent partial tumor resection, chemotherapy, and autologous blood stem cell transplantation. After treatment, the lesion rapidly regressed in the serial follow-up imaging without recurrence.