scRNA-seq in medulloblastoma shows cellular heterogeneity and lineage expansion support resistance to SHH inhibitor therapy

Nat Commun. 2019 Dec 20;10(1):5829. doi: 10.1038/s41467-019-13657-6.

Abstract

Targeting oncogenic pathways holds promise for brain tumor treatment, but inhibition of Sonic Hedgehog (SHH) signaling has failed in SHH-driven medulloblastoma. Cellular diversity within tumors and reduced lineage commitment can undermine targeted therapy by increasing the probability of treatment-resistant populations. Using single-cell RNA-seq and lineage tracing, we analyzed cellular diversity in medulloblastomas in transgenic, medulloblastoma-prone mice, and responses to the SHH-pathway inhibitor vismodegib. In untreated tumors, we find expected stromal cells and tumor-derived cells showing either a spectrum of neural progenitor-differentiation states or glial and stem cell markers. Vismodegib reduces the proliferative population and increases differentiation. However, specific cell types in vismodegib-treated tumors remain proliferative, showing either persistent SHH-pathway activation or stem cell characteristics. Our data show that even in tumors with a single pathway-activating mutation, diverse mechanisms drive tumor growth. This diversity confers early resistance to targeted inhibitor therapy, demonstrating the need to target multiple pathways simultaneously.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Anilides / pharmacology
  • Anilides / therapeutic use
  • Animals
  • Cell Proliferation / drug effects
  • Cell Proliferation / genetics
  • Cerebellar Neoplasms / drug therapy
  • Cerebellar Neoplasms / genetics*
  • Cerebellar Neoplasms / pathology
  • Cerebellum / cytology
  • Cerebellum / pathology
  • Drug Resistance, Neoplasm / genetics*
  • Female
  • Gain of Function Mutation
  • Hedgehog Proteins / antagonists & inhibitors*
  • Hedgehog Proteins / genetics
  • Humans
  • Male
  • Medulloblastoma / drug therapy
  • Medulloblastoma / genetics*
  • Medulloblastoma / pathology
  • Mice
  • Mice, Transgenic
  • Molecular Targeted Therapy / methods
  • MyoD Protein / genetics
  • Neoplastic Stem Cells / drug effects
  • Pyridines / pharmacology
  • Pyridines / therapeutic use
  • RNA-Seq
  • Signal Transduction / drug effects
  • Signal Transduction / genetics*
  • Single-Cell Analysis
  • Smoothened Receptor / genetics
  • Transcription Factor HES-1 / genetics

Substances

  • Anilides
  • Hedgehog Proteins
  • Hes1 protein, mouse
  • HhAntag691
  • MyoD Protein
  • MyoD1 myogenic differentiation protein
  • Pyridines
  • SHH protein, human
  • Shh protein, mouse
  • Smo protein, mouse
  • Smoothened Receptor
  • Transcription Factor HES-1
  • HES1 protein, human