ENETS Centre of Excellence, Liverpool University Hospitals, NHS Foundation Trust, UK.
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.
Eur J Surg Oncol. 2021 Jun;47(6):1332-1338. doi: 10.1016/j.ejso.2020.09.012. Epub 2020 Sep 16.
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.
We retrospectively assessed the impact of lymph nodes and CRH on outcomes, including survival, in all aNET patients diagnosed between 1990 and 2016.
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).
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.
欧洲神经内分泌肿瘤学会（ENETS）建议通过阑尾切除术和可能的右半结肠切除术（CRH）来治疗阑尾神经内分泌肿瘤（aNET）。然而，疾病的行为和生存模式仍然不确定。
我们回顾性评估了淋巴结和 CRH 对所有 1990 年至 2016 年间诊断为 aNET 的患者结局的影响，包括生存。
102 例患者（52 例女性，50 例男性），中位年龄为 39.4 岁（范围 16.3-81.1 岁），被诊断为 aNET。肿瘤平均大小为 12.7 毫米（范围 1-60 毫米），最常见于阑尾尖端（63%）。79%的病例行阑尾切除术作为初始手术，其余患者行结肠切除术。在指数手术后 3.2 个月（范围 1.4-9.8 个月）中位数时对 30 例患者进行了 CRH，其中 9 例有残留疾病：淋巴结（n=8）或残留肿瘤（n=1）。单变量逻辑回归显示，肿瘤大小≥2 厘米（p=0.020）显著预测残留疾病。有 4 例患者拒绝行 CRH，但未发生复发或生存时间缩短。1 例患者在随访 16.5 年后复发，另 1 例患者在随访 18.8 年后发生第二例神经内分泌肿瘤。共有 5 例死亡；1 例与 aNET 相关。5 年和 10 年总生存率分别为 99%和 92%；5 年和 10 年无复发生存率分别为 98%和 92%。只有 5 年无复发生存率受 ENETS 分期影响（p=0.002）。
aNET 具有惰性，总体生存率和无复发生存率非常高。复发罕见，在本系列中仅在数十年后发生，因此强烈支持选择性监测和随访。阳性淋巴结的意义和行右半结肠切除术的必要性仍不清楚。
