Biomedicine

Brainstem Tumor Development

+ See all authors and affiliations

Science  25 Mar 2011:
Vol. 331, Issue 6024, pp. 1493
DOI: 10.1126/science.331.6024.1493-a
CREDIT: MONJE ET AL., PROC. NATL. ACAD. SCI. U.S.A. 108, 10.1073/PNAS.1101657108 (2011). (DIPG IMAGE) MORGAN FRERET AND PHIL BEACHY

Brain cancer is the most common solid tumor in children. For children with medulloblastoma, survival rates have steadily improved as a result of optimized therapies. In contrast, children with an aggressive brainstem tumor called DIPG (for diffuse intrinsic pontine glioma) are far less fortunate, with death occurring usually within a year. Because biopsy specimens of human DIPG are rare and because there are no relevant animal models, little is known about the cellular and molecular origins of these tumors.

A study by Monje et al. provides insight both into the likely cell of origin of DIPG and into a signaling pathway that may help promote tumor growth. The culprit cell appears to be a previously uncharacterized neural precursor cell in the normal human brainstem. The density of these cells peaks during the time of childhood, when DIPGs most commonly arise. In a cell culture model, human DIPG cells (above, overlaid on an MRI scan from a DIPG patient) showed activation of the Hedgehog (Hh) signaling pathway, which is critical to normal brain development and which is aberrantly activated in other human cancers, including medulloblastoma. Thus, DIPG probably arises through dysregulation of postnatal neurodevelopment, and the Hh signaling pathway may be a possible therapeutic target for this tumor.

Proc. Natl. Acad. Sci. U.S.A. 108, 10.1073/pnas.1101657108 (2011).

Navigate This Article