A novel, diffusely infiltrative xenograft model of human anaplastic oligodendroglioma with mutations in FUBP1, CIC, and IDH1

PLoS One. 2013;8(3):e59773. doi: 10.1371/journal.pone.0059773. Epub 2013 Mar 19.

Abstract

Oligodendroglioma poses a biological conundrum for malignant adult human gliomas: it is a tumor type that is universally incurable for patients, and yet, only a few of the human tumors have been established as cell populations in vitro or as intracranial xenografts in vivo. Their survival, thus, may emerge only within a specific environmental context. To determine the fate of human oligodendroglioma in an experimental model, we studied the development of an anaplastic tumor after intracranial implantation into enhanced green fluorescent protein (eGFP) positive NOD/SCID mice. Remarkably after nearly nine months, the tumor not only engrafted, but it also retained classic histological and genetic features of human oligodendroglioma, in particular cells with a clear cytoplasm, showing an infiltrative growth pattern, and harboring mutations of IDH1 (R132H) and of the tumor suppressor genes, FUBP1 and CIC. The xenografts were highly invasive, exhibiting a distinct migration and growth pattern around neurons, especially in the hippocampus, and following white matter tracts of the corpus callosum with tumor cells accumulating around established vasculature. Although tumors exhibited a high growth fraction in vivo, neither cells from the original patient tumor nor the xenograft exhibited significant growth in vitro over a six-month period. This glioma xenograft is the first to display a pure oligodendroglioma histology and expression of R132H. The unexpected property, that the cells fail to grow in vitro even after passage through the mouse, allows us to uniquely investigate the relationship of this oligodendroglioma with the in vivo microenvironment.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Base Sequence
  • DNA Fingerprinting
  • DNA Helicases / genetics*
  • DNA Primers / genetics
  • DNA-Binding Proteins / genetics*
  • Disease Models, Animal*
  • Green Fluorescent Proteins / metabolism
  • Humans
  • Immunohistochemistry
  • Isocitrate Dehydrogenase / genetics*
  • Magnetic Resonance Imaging
  • Mice
  • Mice, Inbred NOD
  • Mice, SCID
  • Microarray Analysis
  • Molecular Sequence Data
  • Oligodendroglioma / genetics*
  • RNA-Binding Proteins
  • Repressor Proteins / genetics*
  • Sequence Analysis, DNA
  • Transplantation, Heterologous

Substances

  • CIC protein, human
  • DNA Primers
  • DNA-Binding Proteins
  • FUBP1 protein, human
  • RNA-Binding Proteins
  • Repressor Proteins
  • enhanced green fluorescent protein
  • Green Fluorescent Proteins
  • Isocitrate Dehydrogenase
  • IDH1 protein, human
  • DNA Helicases

Grants and funding

JMN is supported by the Norwegian Cancer Society (PK01-2009-0349 to JMN). The study was further supported by Helse-Vest, the Haukeland University Hospital, Fonds National Recherche, Luxembourg, and the Centre de Recherche Public de la Santé, Luxembourg. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.