Abstract
Diffuse intrinsic pontine glioma (DIPG) is a universally fatal tumor of the brainstem, most commonly affecting young children. Due to its location, surgical resection is not achievable, but consideration of a biopsy has become standard practice at children’s hospitals with the appropriate neurosurgical expertise. While the decision to obtain a biopsy should be directed by the presence of atypical radiographic features that call the diagnosis of DIPG into question or the requirement of biopsy tissue for clinical trial enrollment, once this precious tissue is available its use for research should be considered. The majority of DIPG and diffuse midline glioma, H3 K27M-mutant (DMG) models are autopsy-derived or genetically-engineered, each of which has limitations for translational studies, so the use of biopsy tissue for laboratory model development provides an opportunity to create unique model systems. Here, we present a detailed laboratory protocol for the generation of treatment-naïve biopsy-derived DIPG/DMG models.
Highlights
Diffuse intrinsic pontine glioma (DIPG) is an aggressive brain tumor that arises in the ventral pons during middle childhood
Clinical trials of radiation-sensitizing agents, intensive chemotherapies, maintenance chemotherapies, and targeted agents have yet to make a significant impact though some conventional chemotherapy regimens may modestly improve and extend life [2,3]
While pontine DMG are often described as having a “classic” imaging appearance, there are inconsistencies in imaging interpretations [29]
Summary
Diffuse intrinsic pontine glioma (DIPG) is an aggressive brain tumor that arises in the ventral pons during middle childhood. Due to its diffuse growth through critical brainstem structures, DIPG is unresectable. The standard of care is limited to focal radiation, most commonly to ~54 Gy, which often improves symptoms and extends life by ~3 months [4]. Considering the unresectable pattern of growth and lack of long-term survivors, DIPG is often diagnosed by imaging alone. It must only be performed by experienced neurosurgeons because transient, though sometimes permanent, neurologic deficits can result [6]. This constellation resulted in decades of extremely limited DIPG tissue for study
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