Background/Objectives: Appendiceal neuroendocrine neoplasms (ANENs) are usually found incidentally during histology examination after appendectomy for appendicitis. Due to their rarity in pediatric populations, there is no consensus on treatment or follow-up. The analysis of patients with ANENs of our and other studies will increase the understanding of this tumor. Methods: Pediatric patients with ANENs were uniformly managed at our center between 1998 and 2023. Patients’ presenting symptoms, surgery, tumor histology, post-surgical work-up, follow-up and outcome were analyzed. Results: Our report describes 17 patients with a diagnosis of ANEN after appendectomy. The median age was 14 years (range of 4–17 years). Tumors were located at the tip of the appendix in 58.8% of cases and only one had a diameter >1 cm. All were well-differentiated tumors with free resection margins. The submucosa was invaded in five cases, muscularis propria in eight and subserosa in four. Post-appendectomy work-up included tumor marker measurement, abdominal ultrasound and computed tomography or magnetic resonance imaging, chest X-ray and octreotide scintigraphy. No residual tumors or metastases were detected. Additional surgery was not necessary. Follow-up was carried out for a median duration of 6 years (range of 1–10 years). Only one patient was lost to follow-up and all other patients are alive without tumor recurrence. Conclusions: The tumor characteristics of our patients confirmed data from the literature. With the lack of a sufficient number of large prospective trials, it is important to add more information to confirm the benign nature and excellent outcome of this tumor, even without additional surgery. Consensus guidelines are needed for ANENs in pediatric populations.
背景/目的：阑尾神经内分泌肿瘤通常在因阑尾炎行阑尾切除术后组织学检查中被偶然发现。由于其在儿童群体中较为罕见，目前对于治疗及随访尚无共识。通过分析本研究及其他研究中ANENs患者的情况，将有助于增进对该肿瘤的认识。方法：1998年至2023年间，本中心对儿童ANENs患者实施标准化诊疗。对患者的临床症状、手术情况、肿瘤组织学特征、术后检查、随访及预后进行系统性分析。结果：本研究报告了17例阑尾切除术后确诊为ANENs的病例。患者中位年龄为14岁（年龄范围4-17岁）。58.8%的肿瘤位于阑尾尖端，仅1例肿瘤直径＞1厘米。所有肿瘤均为高分化型且切缘阴性。其中5例侵犯黏膜下层，8例侵犯固有肌层，4例侵犯浆膜下层。术后检查包括肿瘤标志物检测、腹部超声、计算机断层扫描或磁共振成像、胸部X线及奥曲肽显像。未发现残留肿瘤或转移病灶。所有病例均无需追加手术。中位随访时间为6年（范围1-10年）。除1例失访外，其余患者均存活且无肿瘤复发。结论：本组患者的肿瘤特征与文献报道数据相符。鉴于目前缺乏足够数量的大型前瞻性研究，补充更多临床资料以证实该肿瘤的良性本质及良好预后（即使未行追加手术）具有重要意义。亟需建立针对儿童ANENs的共识指南。
肿瘤(癌症)患者之家