Treatment landscape of advanced high-grade neuroendocrine neoplasms

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...

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Published inClinical advances in hematology & oncology Vol. 21; no. 1; pp. 16 - 26
Main Authors Alheraki, Samee Zane, Almquist, Daniel R, Starr, Jason S, Halfdanarson, Thorvardur R, Sonbol, Mohamad Bassam
Format Journal Article
LanguageEnglish
Published United States 01.01.2023
Subjects
Carcinoma, Neuroendocrine - pathology
Humans
Intestinal Neoplasms - pathology
Neoplasm Grading
Neuroendocrine Tumors - diagnosis
Neuroendocrine Tumors - genetics
Neuroendocrine Tumors - therapy
Pancreatic Neoplasms - drug therapy
Stomach Neoplasms - pathology
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Summary: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.
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ISSN:1543-0790