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Treatment landscape of advanced high-grade neuroendocrine neoplasms
Samee Zane Alheraki1, Daniel R Almquist2, Jason S Starr3
1Division of Hematology and Medical Oncology, Mayo Clinic, Phoenix, Arizona.
Abstract:
Grade 3 neuroendocrine neoplasms (NEN G3) are high-grade (Ki-67 index >20%) neuroendocrine malignancies that comprise both rapidly proliferating, well-differentiated neuroendocrine tumors (NET G3) and poorly differentiated neuroendocrine carcinomas (NEC). The phenotypic differences between NET G3 and NEC stem from differences in their underlying genomic alterations. As a result of these differences, NET G3 is molecularly, radiologically, and prognostically distinct from NEC. The optimal management of NET G3 and NEC is currently being refined through clinical trials that focus on NET G3 and NEC as separate entities. This review aims to summarize the current understanding of NEN G3 by distinguishing between NET G3 and NEC and describing the clinical implications associated with each.
Insights
Grade 3 neuroendocrine neoplasms (NEN G3) include well-differentiated neuroendocrine tumors (NET G3) and poorly differentiated neuroendocrine carcinomas (NEC). These distinct entities have different genomic alterations, impacting their management and prognosis.
Area of Science:
- Oncology
- Pathology
- Genetics
Background:
- Grade 3 neuroendocrine neoplasms (NEN G3) are high-grade malignancies with Ki-67 index >20%.
- NEN G3 encompass both well-differentiated neuroendocrine tumors (NET G3) and poorly differentiated neuroendocrine carcinomas (NEC).
- Phenotypic, genomic, and prognostic differences exist between NET G3 and NEC.
Purpose of the Study:
- To review and summarize the current understanding of NEN G3.
- To differentiate between NET G3 and NEC based on their distinct characteristics.
- To describe the clinical implications of these distinctions for patient management.
Main Methods:
- Literature review of current research on NEN G3.
- Analysis of genomic alterations differentiating NET G3 and NEC.
- Synthesis of radiological and prognostic data for both entities.
Main Results:
- NET G3 and NEC exhibit distinct underlying genomic alterations.
- These molecular differences lead to significant variations in radiological presentation and patient prognosis.
- Current clinical trials are increasingly treating NET G3 and NEC as separate disease entities.
Conclusions:
- Distinguishing between NET G3 and NEC is crucial for accurate diagnosis and treatment planning.
- Understanding the molecular basis of NEN G3 subtypes informs personalized therapeutic strategies.
- Further research and clinical trials focusing on these distinct entities will refine optimal management approaches.
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