Retrospective study on loco-regional digestive neuroendocrine carcinoma

Level: Level 2

Launch date: 1 March 2024

Principle investigators: Halfdan SORBYE

Project description:

Background

Digestive neuroendocrine carcinoma (NEC) is a rare entity with an aggressive growth pattern and frequently characterised by early, extensive metastatic disease. The prognosis for patients with digestive NEC is generally poor despite recent advances in both diagnostics and treatment. Long-term relapse-free survival is however possible among patients with loco-regional disease (stage I-III) after multimodality therapy.

There are conflicting retrospective data on how to treat loco-regional digestive NEC. There is no international consensus on the use of surgery. Furthermore, there is no strong consensus in relation to adjuvant chemotherapy, neoadjuvant chemotherapy or chemoradiation. Older studies do not separate between NET G3 and NEC among high-grade (HG) digestive NEN, and data for NEC alone are needed. The use of pre-operative PET or neoadjuvant chemotherapy might better select patients that may benefit from surgery, but data are lacking. A prospective trial randomising between surgery vs no surgery is not ethical in Europe, and an adjuvant/neoadjuvant randomised trial not feasible due to the rarity of the disease.

A way to move forward in this field is to collect high-quality retrospective data with a high number of patients. We propose a study on this using high-quality data from selected NET centres within ENETS, using the present ENETS database.

Objectives

To characterise diagnosis and treatment of locoregional (stage I-III) digestive neuroendorine carcinoma patients, specifying

• patient charateristics: age, gender, co-morbidity (Cumulative Illness Rating Scale), ECOG performance status, etc
• tumor characteristics: primary tumor location, Ki-67, morphology, mutation status if available etc
• diagnostic procedures: Use of pre-operative FDG-PET
• surgical treatment of primary tumor
• chemotherapy treatment (adjuvant and neoadjuvant)
• use of chemoradiation
• recurrence, time to recurrence, site of recurrence, predictive factors for recurrence
• survival, prognostic factors for survival

Inclusion criteria

• Histological proven neuroendocrine carcinoma
• Initial stage I-III.
• Primary digestive tumor site
• Initial diagnosis 2012-2021
• Cases with Ki-67 < 55% must have been re-evaluated according to the 2019 WHO classification.

Exclusion criteria

• NET G3 are excluded.
• MiNEN are excluded.

Study design and plan

A retrospective multi-centre study in patients with loco-regional (Stage I-III) digestive neuroendocrine carcinoma. The study is planned to evaluate >150 eligible patients. Only selected centers are invited to participate in the study.

Publication rules

The study will be published once it is completed, and the final analysis has been performed. The names on the author list will be given mainly according to the recruitment of eligible patients, but also in a minor part if a major contribution was made in the design of the protocol, in the compilation of results and in the production of the article decided by the principle investigator. Being a part of the study protocol commitee does not by itself qualify for co-authorship.

Protocol committee

• Halfdan Sorbye
• Mairéad McNamara
• Thomas Walter
• Rocio Garcia-Carbonero
• Margot Tesselaar
• Marianne Pavel
• Julian Haddoux
• Seppo W. Langer
• Anna Pellat
• Ulrich Knigge
• Eva Tiensu Janson
• Nicola Fazio