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

TL;DR

This study examines the treatment and outcomes of appendix neuroendocrine tumors, finding that most cases can be managed with surgery alone and may not require routine follow-up.

Contribution

The study provides insights into optimal management and surveillance strategies for appendiceal neuroendocrine neoplasms based on a multicenter international cohort.

Findings

Most aNENs are effectively managed with appendectomy alone.

Routine follow-up may be unnecessary without adverse pathological features.

Right hemicolectomies were more frequent in the Irish cohort compared to the Italian cohort.

Abstract

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.

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