Abstract
Diffuse intrinsic pontine glioma (DIPG) is one of the least understood and most deadly childhood cancers. Historically, there has been a paucity of DIPG specimens for molecular analysis. However, due to the generous participation of DIPG families in programs for postmortem specimen donation, there has been a recent surge in molecular analysis of newly available tumor specimens. Collaborative efforts to share data and tumor specimens have resulted in rapid discoveries in other pediatric brain tumors, such as medulloblastoma, and therefore have the potential to shed light on the biology of DIPG. Given the generous gift of postmortem tissue donation from DIPG patients, there is a need for standardized postmortem specimen accrual to facilitate rapid and effective multi-institutional molecular studies.We developed and implemented an autopsy protocol for rapid procurement, documenting and storing these specimens. Sixteen autopsies were performed throughout the United States and Canada and processed using a standard protocol and inventory method, including specimen imaging, fixation, snap freezing, orthotopic injection, or preservation. This allowed for comparative clinical and biological studies of rare postmortem DIPG tissue specimens, generation of in vivo and in vitro models of DIPG, and detailed records to facilitate collaborative analysis.
Highlights
Diffuse intrinsic pontine glioma (DIPG) is one of the most poorly understood childhood cancers
After decades of stagnation in the study of DIPG biology there has been a recent surge in molecular profiling of DIPG tumor tissue, resulting in identification of novel mutations and genomic aberrations in this lethal cancer [1,2,3,4,5]
Recent identification of Histone 3 K27M mutation and other www.impactjournals.com/oncotarget unique genomic aberrations driving DIPG were made possible by the availability of autopsied and rare biopsied specimens for molecular analysis [2, 4, 5, 8, 9]
Summary
Diffuse intrinsic pontine glioma (DIPG) is one of the most poorly understood childhood cancers. Due to the neuroanatomical location of DIPG and its infiltrative nature, this tumor is not amenable to surgical resection. The lack of readily available surgical specimens, coupled with the progressive nature and high morbidity of this disease has led to a paucity of specimens for molecular studies. After decades of stagnation in the study of DIPG biology there has been a recent surge in molecular profiling of DIPG tumor tissue, resulting in identification of novel mutations and genomic aberrations in this lethal cancer [1,2,3,4,5]. One of the main factors contributing to this expanding knowledge of DIPG biology is the selfless gift from children with DIPG and their families of postmortem www.impactjournals.com/oncotarget tumor donation for biological studies. Despite some efforts to implement a standardized autopsy protocol for specimen acquisition [6], there is a continued need to improve postmortem procurement procedures to ensure optimized use of these precious specimens across North America and Europe, within DIPG collaborative groups and consortia
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