Henderson Lucy, Fehily Claere, Folaranmi Semiu, Kelsey Anna, McPartland Jo, Jawaid Wajid B, Craigie Ross, Losty Paul D
Department of Paediatric Surgery, Royal Manchester Children's Hospital, Manchester, UK.
Department of Paediatric Surgery, Alder Hey Children's Hospital NHS Foundation Trust and The University of Liverpool, UK.
J Pediatr Surg. 2014 Oct;49(10):1513-7. doi: 10.1016/j.jpedsurg.2014.05.019. Epub 2014 Jun 25.
Neuroendocrine tumours (NET) of the appendix are rare histopathological neoplasms detected following operation for appendicitis in childhood. The role (if any) for radical surgery notably right hemicolectomy (RHC) has often reflected the 'expert opinion' of adult general surgeons with wider experience of managing NET lesions of the gastrointestinal tract. Critical decisions have focused on (a) tumour size, (b) histology, (c) tumour location/invasion and (d) positive lymph nodes. Against this background we report the clinical outcome of children with 'incidental' appendix carcinoid tumours managed at two regional UK paediatric surgery centres. A critical review of the literature is additionally provided in an effort to define contemporary patterns of care in paediatric surgical practice.
Hospital records and pathology database(s) identified 27 patients at two UK centres with a confirmed histological diagnosis of appendix NET lesions during January 1997-January 2013. A PUBMED and EMBASE search strategy-(English language publications only), 1975-present, was performed to gather information on all patients younger than 20years at primary diagnosis with NET appendix tumours to review their management and outcomes.
All 27 patients treated at the two institutions had acute appendicitis including 3 cases presenting with an appendix mass. Twenty-five underwent appendicectomy with two having interval operations. Tumours had a maximum diameter of 2-18mm (median 9mm) with 73% of lesions located at the appendix tip. Fourteen (52%) had tumour invading the mesoappendix. All patients underwent appendicectomy only with no single case having RHC or additional surgery. Surveillance studies (5HIAA, chromogranin-A) and imaging including ultrasound or CT were deployed in a minority of patients revealing no abnormality. All 27 cases are alive and well-(mean follow up 5years; range: 9months-16years). The literature highlights varied management strategies and no recorded fatalities with radical surgery in children largely evolving from adult surgical practice.
This study confirms that paediatric patients with 'incidental' NET tumours of the appendix have an excellent prognosis. Consensus guidelines should ideally be developed by paediatric oncology surgeons to avoid unnecessary radical surgery in many otherwise healthy children.
阑尾神经内分泌肿瘤（NET）是一种罕见的组织病理学肿瘤，在儿童阑尾炎手术后被发现。根治性手术尤其是右半结肠切除术（RHC）的作用（如果有）常常反映了在管理胃肠道NET病变方面经验更丰富的成人普通外科医生的“专家意见”。关键决策集中在（a）肿瘤大小、（b）组织学、（c）肿瘤位置/侵犯情况和（d）阳性淋巴结。在此背景下，我们报告了在英国两个地区儿科手术中心接受治疗的“偶然发现的”阑尾类癌肿瘤患儿的临床结果。此外，对文献进行了批判性综述，以确定儿科外科实践中的当代护理模式。
通过医院记录和病理数据库，在英国两个中心确定了27例在1997年1月至2013年1月期间经组织学确诊为阑尾NET病变的患者。采用PUBMED和EMBASE检索策略（仅检索英文出版物，时间范围为1975年至今），收集所有初次诊断为阑尾NET肿瘤时年龄小于20岁患者的信息，以回顾他们的治疗和结局。
在这两个机构接受治疗的所有27例患者均患有急性阑尾炎，其中3例表现为阑尾肿块。25例行阑尾切除术，2例行间隔手术。肿瘤最大直径为2 - 18毫米（中位数9毫米），73%的病变位于阑尾尖端。14例（52%）肿瘤侵犯阑尾系膜。所有患者仅接受了阑尾切除术，无一例接受RHC或其他手术。少数患者进行了监测研究（5 - HIAA、嗜铬粒蛋白A）以及包括超声或CT在内的影像学检查，未发现异常。所有27例患者均存活且状况良好（平均随访5年；范围：9个月至16年）。文献强调了不同的治疗策略，且儿童根治性手术无死亡记录，这在很大程度上源于成人外科实践。
本研究证实，患有“偶然发现的”阑尾NET肿瘤的儿科患者预后良好。理想情况下，儿科肿瘤外科医生应制定共识指南，以避免在许多其他方面健康的儿童中进行不必要的根治性手术。
