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Updated: Mar 1, 2026

Author Spotlight: Genetically Engineered Mouse Models and Pathological Characterization of Neurofibromatosis Type 1 Associated Tumors
Published on: May 17, 2024
Biology and evolution of poorly differentiated neuroendocrine tumors
David S Rickman1,2,3, Himisha Beltran1,3,4, Francesca Demichelis1,5,6
1Caryl and Israel Englander Institute for Precision Medicine, New York Presbyterian Hospital-Weill Cornell Medicine, New York, New York, USA.
Abstract:
Neuroendocrine (NE) cancers are a diverse group of neoplasms typically diagnosed and treated on the basis of their site of origin. This Perspective focuses on advances in our understanding of the tumorigenesis and treatment of poorly differentiated neuroendocrine tumors. Recent evidence from sequencing indicates that, although neuroendocrine tumors can arise de novo, they can also develop as a result of lineage plasticity in response to pressure from targeted therapies. We discuss the shared genomic alterations of these tumors independently of their site of origin, and we explore potential therapeutic strategies on the basis of recent biological findings.
Insights
Poorly differentiated neuroendocrine tumors can arise anew or from lineage plasticity due to targeted therapies. Understanding shared genomic alterations is key to developing novel treatments for these diverse cancers.
Area of Science:
- Oncology
- Genomics
- Cancer Biology
Background:
- Neuroendocrine (NE) cancers are diverse neoplasms often classified by their primary site.
- Poorly differentiated neuroendocrine tumors represent a challenging subset requiring further understanding.
Purpose of the Study:
- To review advances in the tumorigenesis and treatment of poorly differentiated neuroendocrine tumors.
- To explore shared genomic alterations and potential therapeutic strategies.
Main Methods:
- Review of recent sequencing evidence.
- Analysis of genomic alterations across different tumor origins.
- Exploration of biological findings for therapeutic insights.
Main Results:
- Neuroendocrine tumors can arise de novo or develop via lineage plasticity under therapeutic pressure.
- Shared genomic alterations are observed in these tumors irrespective of their origin site.
Conclusions:
- Understanding the molecular basis of neuroendocrine tumor development, including lineage plasticity, is crucial.
- Targeting shared genomic alterations presents a promising avenue for novel therapeutic strategies.

