Wnt Signaling in Ewing Sarcoma, Osteosarcoma, and Malignant Peripheral Nerve Sheath Tumors

Curr Osteoporos Rep. 2017 Aug;15(4):239-246. doi: 10.1007/s11914-017-0377-9.

Abstract

Purpose of review: Wnt signaling plays a central role in development and homeostasis, and its dysregulation is a common event in many types of human cancer. Here we explore in detail the contributions of Wnt signaling to the initiation and maintenance of three types of saroma: Ewing sarcoma, osteosarcoma, and malignant peripheral nerve sheath tumors. This review provides an overview of the Wnt signaling pathway and explores in detail the current knowledge about its role in the initiation or maintenance of three tumor types: Ewing sarcoma, osteosarcoma, and malignant peripheral nerve sheath tumors.

Recent findings: Recent work has assessed the role(s) of Wnt signaling within these cell types. This review provides an overview of the mechanistic insights that have been gained from a number of recent studies to set the foundation for potential therapeutic applications. Wnt signaling has emerged as a potentially critical pathway in maintaining the growth of these types of tumors. Given the fact that many new inhibitors of the pathway have recently or will soon enter Phase 1 clinical trials, it is likely that assessment of their activity in these tumor types will occur in human patients.

Keywords: Ewing sarcoma; Malignant peripheral nerve sheath tumors; Neurofibromatosis Type 1; Osteosarcoma; Wnt; β-catenin.

Publication types

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

MeSH terms

  • Bone Neoplasms / metabolism*
  • Humans
  • Neurilemmoma / metabolism*
  • Neurofibromatosis 1 / metabolism
  • Osteosarcoma / metabolism*
  • Sarcoma, Ewing / metabolism*
  • Wnt Proteins / metabolism
  • Wnt Signaling Pathway*
  • beta Catenin / metabolism

Substances

  • Wnt Proteins
  • beta Catenin