Multicenter Retrospective Study of Efficacy of Systemic Therapy in Patients with Neuroendocrine Tumor Grade 3

Hiroyuki Okuyama1, Taro Shibuki2, Naohiro Okano3

  • 1Department of Medical Oncology, Kagawa University Hospital, Miki, Japan, okuyama.hiroyuki@kagawa-u.ac.jp.

Neuroendocrinology
|September 26, 2025
PubMed
Abstract

Insights

Chemotherapy for neuroendocrine tumor G3 (NET G3) shows similar survival outcomes. However, NET-based cytotoxic chemotherapy demonstrated a higher response rate compared to neuroendocrine carcinoma-based treatment.

Area of Science:

  • Oncology
  • Medical Research

Background:

  • Neuroendocrine tumor G3 (NET G3) is a recently defined entity in the WHO 2017 classification.
  • Limited evidence exists regarding optimal chemotherapy for NET G3.
  • This study evaluates systemic therapy outcomes for NET G3.

Purpose of the Study:

  • To assess the efficacy of different chemotherapy regimens for NET G3.
  • To identify optimal treatment strategies for NET G3 patients.

Main Methods:

  • Retrospective analysis of 73 NET G3 patients treated between 2011-2019.
  • Evaluation of patient demographics, chemotherapy regimens, and treatment outcomes.
  • Central pathological review for 44 patients.

Main Results:

  • No significant difference in progression-free survival (PFS) or overall survival (OS) between NET-based and neuroendocrine carcinoma (NEC)-based treatments.
  • Response rate (RR) was higher for NET-based cytotoxic chemotherapy (50%) versus NEC-based treatment (16%).
  • Pancreatic NETs comprised the largest subgroup (47 patients).

Conclusions:

  • While PFS and OS did not differ significantly across regimens, NET-based cytotoxic chemotherapy showed a superior response rate.
  • Further research into optimal NET G3 chemotherapy is warranted.