Gastroenteropancreatic grade 3 neuroendocrine tumors: a single entity or a heterogeneous group? A retrospective analysis

J Endocrinol Invest. 2022 Feb;45(2):317-325. doi: 10.1007/s40618-021-01642-0. Epub 2021 Jul 19.

Abstract

Purpose: Grade 3 neuroendocrine tumor (NET G3) is a novel pathologic category within gastro-entero-pancreatic (GEP) neuroendocrine neoplasms (NENs) but its clinical behavior and therapeutic management still remain challenging. Prognostic and predictive factors aiding NET G3 management are needed.

Patients and methods: We performed a retrospective analysis from 2015 to 2020 of all patients with > 20% Ki-67, well-differentiated NETs evaluated within our NEN-dedicated multidisciplinary team. We divided the sample according the timing of NET G3 diagnosis, the radiotracers distribution and Ki-67. We analyzed the correlation between these NET G3 features and clinical outcomes.

Results: Among 3238 multidisciplinary discussion reports, we selected 55 patients, 48 from GEP and 7 from an occult GEP origin. In 45 patients, NET G3 diagnosis occurred at the beginning of clinical history (upfront-NET G3), whereas in 10, during the NET G1-G2 clinical history (late-NET G3). Patients with ≤ 30% (34/55) vs. > 30% Ki-67 (21/55) had a better overall survival (OS) (p = 0.042); patients with a homogeneous vs. inhomogeneous/negative 68Gallium(68Ga)-DOTA-Peptide Positron Emission Tomography (PET)/computed tomography (CT) showed a trend to a better OS, and a significant better progression-free survival (PFS) (p = 0.033). A better OS was observed for negative/inhomogeneous vs. homogeneous 18-fluorodeoxyglucose (18FDG)-PET/CT (p = 0.027). A trend to a better OS was reported in late- vs. upfront-NET G3, while the latter showed a significantly better response rate (RR) (p = 0.048).

Conclusion: Our findings suggested that Ki-67 cutoff, functional imaging and the timing to NET G3 diagnosis may help clinicians in more accurate selection of NET G3 management. Prospective studies are needed.

Keywords: G3; NENs; NETs; Neuroendocrine.

MeSH terms

  • Early Detection of Cancer / methods*
  • Female
  • Fluorodeoxyglucose F18 / pharmacology
  • Humans
  • Immunohistochemistry
  • Intestinal Neoplasms* / diagnosis
  • Intestinal Neoplasms* / metabolism
  • Intestinal Neoplasms* / mortality
  • Intestinal Neoplasms* / therapy
  • Italy / epidemiology
  • Ki-67 Antigen / analysis*
  • Male
  • Middle Aged
  • Neoplasm Grading
  • Neuroendocrine Tumors* / diagnosis
  • Neuroendocrine Tumors* / metabolism
  • Neuroendocrine Tumors* / mortality
  • Neuroendocrine Tumors* / therapy
  • Organometallic Compounds / pharmacology
  • Pancreatic Neoplasms* / diagnosis
  • Pancreatic Neoplasms* / metabolism
  • Pancreatic Neoplasms* / mortality
  • Pancreatic Neoplasms* / therapy
  • Patient Selection
  • Positron Emission Tomography Computed Tomography* / methods
  • Positron Emission Tomography Computed Tomography* / statistics & numerical data
  • Prognosis
  • Radiopharmaceuticals / pharmacology
  • Retrospective Studies
  • Stomach Neoplasms* / diagnosis
  • Stomach Neoplasms* / metabolism
  • Stomach Neoplasms* / mortality
  • Stomach Neoplasms* / therapy
  • Survival Analysis

Substances

  • Ki-67 Antigen
  • Organometallic Compounds
  • Radiopharmaceuticals
  • Fluorodeoxyglucose F18
  • gallium Ga 68 dotatate

Supplementary concepts

  • Gastro-enteropancreatic neuroendocrine tumor