Storan Darragh, Swan Niall, Swan Kara, Thuillier Rhona, Skehan Stephen, Gallagher Tom, O'Shea Donal, O'Toole Dermot
National Centre for Neuroendocrine Tumour, St. Vincent's University Hospital, Dublin, Ireland.
Department of Histopathology, St. Vincent's University Hospital, Dublin, Ireland.
J Neuroendocrinol. 2023 Oct;35(10):e13329. doi: 10.1111/jne.13329. Epub 2023 Aug 13.
Appendiceal neuroendocrine tumours (aNETs) are rare neoplasms of the gastrointestinal tract often diagnosed incidentally at the time of appendicectomy. Appendicectomy is considered curative in the majority of cases but guidelines recommend right-sided hemicolectomy (RHC) for those with specific high-risk features despite no data supporting a survival benefit. We performed a retrospective search of multi-disciplinary tumour board and pathology databases from 2012 to 2022 to identify cases of aNET treated at our centre. Follow-up data were obtained from the electronic healthcare records. A total of 142 cases of aNET were included for analysis. Mean age at presentation was 34, of which 76% were female and 92% of aNETs were located in the tip/middle of the appendix; 90% were grade 1, and 93% had R0 resection. Tumour size was <1 cm in 54%, 1-2 cm in 36%, >2 cm in 9%. A total of 43 patients (30%) underwent RHC with lymph node metastases identified in 16 (37%). Lymph node metastases were associated with tumour size >2 cm (p = .008) and higher tumour grade (p = .041) on multivariate analysis. For aNET 1-2 cm, lymph node metastases were identified in 7/22 who had RHC (32%) with tumour grade the only significant risk factor (p = .046). Distant metastases were identified in 2 cases (1%), diagnosed synchronously and associated with grade 2 tumours. Overall survival for those with lymph node metastases was 100% after a median 4 years. Progression-free survival was 93%, with a single case of disease progression associated with synchronous distant metastases at initial diagnosis. Lymph node metastases in aNET are associated with higher tumour grade and tumour size >2 cm. Disease progression in the setting of lymph node metastases is rare. The significance of lymph node metastases and need for completion RHC remains uncertain.
阑尾神经内分泌肿瘤（aNETs）是胃肠道的罕见肿瘤，常在阑尾切除时偶然被诊断出来。在大多数情况下，阑尾切除术被认为是治愈性的，但指南建议，对于具有特定高危特征的患者进行右侧半结肠切除术（RHC），尽管尚无数据支持其能带来生存获益。我们对2012年至2022年的多学科肿瘤委员会和病理数据库进行了回顾性检索，以确定在我们中心接受治疗的aNET病例。随访数据来自电子医疗记录。共纳入142例aNET病例进行分析。就诊时的平均年龄为34岁，其中76%为女性，92%的aNET位于阑尾尖端/中部；90%为1级，93%实现了R0切除。肿瘤大小<1 cm的占54%，1 - 2 cm的占36%，>2 cm的占9%。共有43例患者（30%）接受了RHC，其中16例（37%）发现有淋巴结转移。多因素分析显示，淋巴结转移与肿瘤大小>2 cm（p = 0.008）和更高的肿瘤分级（p = 0.041）相关。对于1 - 2 cm的aNET，在接受RHC的22例中有7例（32%）发现有淋巴结转移，肿瘤分级是唯一的显著危险因素（p = 0.046）。发现2例（1%）有远处转移，为同时诊断，且与2级肿瘤相关。有淋巴结转移患者的总生存率在中位4年后为100%。无进展生存率为93%，仅有1例疾病进展与初始诊断时的同步远处转移相关。aNET中的淋巴结转移与更高的肿瘤分级和肿瘤大小>2 cm相关。淋巴结转移情况下的疾病进展很少见。淋巴结转移的意义以及是否需要完成RHC仍不确定。
