MB3W1 is an orthotopic xenograft model for anaplastic medulloblastoma displaying cancer stem cell- and Group 3-properties.

Sebastian Dietl,Andreas Rosenwald,Matthias Eyrich,Tilmann Schweitzer,Vijay Ramaswamy,Paul G Schlegel,Stefan Rutkowski,Marc Remke,Michael D Taylor,Stefanie Schwinn,Giles H Vince,Frank Deinlein,Andreas Beilhack,Susanne Dietl,Daniel Picard,Matthias Wölfl,Camelia Monoranu ,Jürgen Krauß ,Simone S Riedel ,André O Von Bueren

doi:10.1186/s12885-016-2170-z

Abstract

BackgroundMedulloblastoma is the most common malignant brain tumor in children and can be divided in different molecular subgroups. Patients whose tumor is classified as a Group 3 tumor have a dismal prognosis. However only very few tumor models are available for this subgroup.MethodsWe established a robust orthotopic xenograft model with a cell line derived from the malignant pleural effusions of a child suffering from a Group 3 medulloblastoma.ResultsBesides classical characteristics of this tumor subgroup, the cells display cancer stem cell characteristics including neurosphere formation, multilineage differentiation, CD133/CD15 expression, high ALDH-activity and high tumorigenicity in immunocompromised mice with xenografts exactly recapitulating the original tumor architecture.ConclusionsThis model using unmanipulated, human medulloblastoma cells will enable translational research, specifically focused on Group 3 medulloblastoma.Electronic supplementary materialThe online version of this article (doi:10.1186/s12885-016-2170-z) contains supplementary material, which is available to authorized users.

Highlights

Medulloblastoma is the most common malignant brain tumor in children and can be divided in different molecular subgroups
Magnetic resonance imaging (MRI) revealed a cerebellar tumor arising from the bottom of the 4th ventricle
Immediate tumor resection was performed and the diagnosis was confirmed as anaplastic medulloblastoma (Fig. 1a and b)

Summary

Introduction

Medulloblastoma is the most common malignant brain tumor in children and can be divided in different molecular subgroups. The approach significantly increased survival rates over the last decades, but a subset of tumors with a still devastating prognosis remains. These aggressive tumors do not respond even to high intensity treatment regimens [2]. As Group 3 tumors have the worst prognosis among the identified subgroups, there is a clear need for reliable tumor models. This subgroup of medulloblastoma almost only occurs in infants and children, in males [23, 24]. It is marked by an extremely high dissemination tendency into the cerebrospinal fluid (CSF)

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Discussion

Conclusion