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.

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.