International Review of Ophthalmology

Previous Articles     Next Articles

Research progress of RB1+/+MYCNA retinoblastoma

Li Weiwei, Zhou Xiyuan   

  1. Department of Ophthalmology, the Second Affiliated Hospital of Chongqing Medical University, Chongqing 400010, China
  • Received:2019-09-18 Online:2020-04-22 Published:2020-04-23
  • Contact: Zhou Xiyuan, Email: zhouxiyuan2002@aliyun.com
  • Supported by:

    Key Science and Technology Innovation project of Social Undertakings and People's Livelihood Security in Chongqing（cstc2017shms-zdyfX0021）

Abstract:

Retinoblastoma is the most common malignant ocular tumor among young children. The famous “two-hit hypothesis” states that loss of both alleles of a tumor suppressor gene (RB1 gene) is necessary to initiate retinoblastoma. However, recent researches reveal that oncogene MYCN amplification is much more common among unilateral retinoblastoma without a detectable RB1 mutation, thus RB1+/+MYCNA is regarded as a new genotype of retinoblastoma. Compared to classical RB1-/- tumors, RB1+/+/MYCNA tumors show early onset, strongers and low salvage rate. MYCN gene also provides a new target for precise treatment of retinoblastoma. (Int Rev Ophthalmol, 2020, 44：133-139)