Journal Article

Clinical Characteristics, Management, and Prognostic Factors of Appendiceal Neuroendocrine Neoplasms: Insights from a Multicenter International Study

Samarasinghe, Kasun, Massironi, Sara, Tamagno, Gianluca, Cantù, Paolo, Cavalcoli, Federica, Del Gobbo, Alessandro, Mulligan, Niall, Caimo, Alberto, Rausa, Emanuele

2025

Overview

Introduction: Appendiceal neuroendocrine neoplasms (aNENs) are the most common malignant appendiceal neoplasms. Localized aNENs are typically managed with an appendectomy; however, right colectomy may be necessary in patients with a high risk of nodal disease. However, the role of right hemicolectomy and the optimal surveillance strategy, particularly for tumors between 1 and 2 cm, remains controversial. Material and Methods: This retrospective, observational study evaluated patients diagnosed with aNENs between January 1995 and July 2015 at two tertiary centers in Ireland and Italy. Data were extracted from a prospectively maintained registry and included clinical, pathological, and therapeutic variables, as well as follow-up outcomes. Results: Forty-three patients (41.8% male; median age 27.5 years) were included, with a median follow-up of 49 months. The median tumor size was 6.4 mm (range: 0.6–40 mm). The majority were G1 tumors (58%), and staging distribution was predominantly Stage I (60%). While no significant differences in demographics or tumor features were observed between centers, completion right hemicolectomies were more frequent in the Irish cohort (p = 0.04). Follow-up practices varied, with more intensive imaging and biochemical monitoring observed in the Italian cohort. Overall prognosis was excellent, with a single case of recurrence during the study period. Conclusions: Most aNENs are effectively managed with appendectomy alone, and routine follow-up may be unnecessary in the absence of adverse pathological features. Accurate risk stratification, driven by comprehensive histopathological assessment, is critical for optimizing management and surveillance strategies.

• Publisher: MDPI AG
• Subject: Age / Appendectomy / appendiceal neuroendocrine carcinoma / appendiceal neuroendocrine neoplasm / appendiceal neuroendocrine tumor / Appendicitis / appendix / carcinoid / Development and progression / Health aspects / Lymphatic system / Medical prognosis / Medical research / Medicine, Experimental / Metastasis / Neuroendocrine tumors / Pathology / Patients / Surgery / Surveillance / Tumors