Edward Alabraba1, David Mark Pritchard2, Rebecca Griffin3, Rafael Diaz-Nieto4, Melissa Banks4, Daniel James Cuthbertson5, Stephen Fenwick4. 1. ENETS Centre of Excellence, Liverpool University Hospitals, NHS Foundation Trust, UK. Electronic address: edwardal@liv.ac.uk. 2. ENETS Centre of Excellence, Liverpool University Hospitals, NHS Foundation Trust, UK; Department of Molecular and Clinical Cancer Medicine, Institute of Systems, Molecular and Integrative Biology, University of Liverpool, UK. 3. Liverpool Clinical and Cancer Research, UK Trials Unit, University of Liverpool, UK. 4. ENETS Centre of Excellence, Liverpool University Hospitals, NHS Foundation Trust, UK. 5. ENETS Centre of Excellence, Liverpool University Hospitals, NHS Foundation Trust, UK; Department of Cardiovascular and Metabolic Medicine, Institute of Life Course and Medical Sciences, University of Liverpool, UK.
Abstract
INTRODUCTION: European Neuroendocrine Tumour Society (ENETS) recommends managing appendiceal neuroendocrine tumours (aNET) with appendicectomy and possibly completion right hemicolectomy (CRH). However, disease behaviour and survival patterns remain uncertain. MATERIALS AND METHODS: We retrospectively assessed the impact of lymph nodes and CRH on outcomes, including survival, in all aNET patients diagnosed between 1990 and 2016. RESULTS: 102 patients (52F, 50 M), median age 39.4 (range 16.3-81.1) years, were diagnosed with aNET. Mean tumour size was 12.7 (range 1-60) mm, most sited in appendiceal tip (63%). Index surgery was appendicectomy in 79% of cases while the remainder underwent colectomy. CRH performed in 30 patients at a median 3.2 (range 1.4-9.8) months post-index surgery yielded residual disease in nine: lymph nodes (n = 8) or residual tumour (n = 1). Univariate logistic regression showed residual disease was significantly predicted by tumour size ≥2 cm (p = 0.020). Four patients declined CRH, but did not suffer relapse or reduced survival. One patient developed recurrence after 16.5 years of follow-up and another patient developed a second neuroendocrine tumour after 18.8 years follow-up. There were 5 deaths; one being aNET-related. 5-year and 10-year overall survival were 99% and 92% respectively; 5-year and 10-year relapse-free survival were 98% and 92% respectively. Only 5-year relapse-free survival was affected by ENETS stage (p = 0.002). CONCLUSION: aNETs are indolent with very high rates of overall and relapse-free survival. Recurrence is rare, and in this series only occurred decades later, making a compelling case for selective surveillance and follow-up. The significance of positive lymph nodes and the necessity for completion right hemicolectomy remain unclear.
INTRODUCTION: European Neuroendocrine Tumour Society (ENETS) recommends managing appendiceal neuroendocrine tumours (aNET) with appendicectomy and possibly completion right hemicolectomy (CRH). However, disease behaviour and survival patterns remain uncertain. MATERIALS AND METHODS: We retrospectively assessed the impact of lymph nodes and CRH on outcomes, including survival, in all aNET patients diagnosed between 1990 and 2016. RESULTS: 102 patients (52F, 50 M), median age 39.4 (range 16.3-81.1) years, were diagnosed with aNET. Mean tumour size was 12.7 (range 1-60) mm, most sited in appendiceal tip (63%). Index surgery was appendicectomy in 79% of cases while the remainder underwent colectomy. CRH performed in 30 patients at a median 3.2 (range 1.4-9.8) months post-index surgery yielded residual disease in nine: lymph nodes (n = 8) or residual tumour (n = 1). Univariate logistic regression showed residual disease was significantly predicted by tumour size ≥2 cm (p = 0.020). Four patients declined CRH, but did not suffer relapse or reduced survival. One patient developed recurrence after 16.5 years of follow-up and another patient developed a second neuroendocrine tumour after 18.8 years follow-up. There were 5 deaths; one being aNET-related. 5-year and 10-year overall survival were 99% and 92% respectively; 5-year and 10-year relapse-free survival were 98% and 92% respectively. Only 5-year relapse-free survival was affected by ENETS stage (p = 0.002). CONCLUSION: aNETs are indolent with very high rates of overall and relapse-free survival. Recurrence is rare, and in this series only occurred decades later, making a compelling case for selective surveillance and follow-up. The significance of positive lymph nodes and the necessity for completion right hemicolectomy remain unclear.
Authors: José Luis Muñoz de Nova; Jorge Hernando; Miguel Sampedro Núñez; Greissy Tibisay Vázquez Benítez; Eva María Triviño Ibáñez; María Isabel Del Olmo García; Jorge Barriuso; Jaume Capdevila; Elena Martín-Pérez Journal: World J Gastroenterol Date: 2022-04-07 Impact factor: 5.374