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Review
. 2017 Sep;4(3):138-150.
doi: 10.1093/nop/npx011. Epub 2017 Aug 11.

Case-based review: pediatric medulloblastoma

Cassie N Kline  1 , Roger J Packer  1 , Eugene I Hwang  1 , David R Raleigh  1 , Steve Braunstein  1 , Corey Raffel  1 , Pratiti Bandopadhayay  1 , David A Solomon  1 , Mariam Aboian  1 , Soonmee Cha  1 , Sabine Mueller  1
Affiliations
Review

Case-based review: pediatric medulloblastoma

Cassie N Kline et al. Neurooncol Pract. 2017 Sep.

Abstract

Medulloblastoma is the most common malignant brain tumor affecting children. These tumors are high grade with propensity to metastasize within the central nervous system and, less frequently, outside the neuraxis. Recent advancements in molecular subgrouping of medulloblastoma refine diagnosis and improve counseling in regards to overall prognosis. Both are predicated on the molecular drivers of each subgroup-WNT-activated, SHH-activated, group 3, and group 4. The traditional therapeutic mainstay for medulloblastoma includes a multimodal approach with surgery, radiation, and multiagent chemotherapy. As we discover more about the molecular basis of medulloblastoma, efforts to adjust treatment approaches based on molecular risk stratification are under active investigation. Certainly, the known neurological, developmental, endocrine, and psychosocial injury related to medulloblastoma and its associated therapies motivate ongoing research towards improving treatment for this life-threatening tumor while at the same time minimizing long-term side effects.

Keywords: chemotherapy; medulloblastoma; radiation; risk stratification; targeted therapy.

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Figures

Fig. 1
Fig. 1
Axial (A) and sagittal (B) postgadolinium, T1-weighted images at time of diagnosis.
Fig. 2
Fig. 2
Axial (A) and sagittal (B) postgadolinium, T1-weighted images postsurgical resection.
Fig. 3
Fig. 3
Axial fluid-attenuated inversion recovery (FLAIR) imaging of brain (A), postgadolinium, T1-weighted lumbar spine (B), and cervical and thoracic spine (C) at time of recurrence. Arrows indicate sites of recurrent, metastatic disease.
Fig. 4
Fig. 4
Pathology from initial diagnosis showing diffuse positivity for GAB1 staining (A), scattered nodules (B), reticulin (C), and desmoplasia (D).
Fig. 5
Fig. 5
Example of World Health Organization (WHO) 2016 molecularly integrated classification for medulloblastoma, with anticipated corresponding clinical and prognostic characteristics.,
See this image and copyright information in PMC

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