Paediatrics and Adolescent Medicine, Faculty of Medicine, University of Augsburg, Augsburg, Germany.
Paediatrics and Adolescent Medicine, Faculty of Medicine, University of Augsburg, Augsburg, Germany; Department of Paediatrics, Paediatric Haematology/Oncology, Otto-von-Guericke-University, Magdeburg, Germany.
Eur J Surg Oncol. 2024 Apr;50(4):108051. doi: 10.1016/j.ejso.2024.108051. Epub 2024 Feb 27.
Paediatric appendiceal neuroendocrine tumours (appNET) are very rare tumours, mostly detected incidentally by histopathological evaluation after appendectomy. Treatment recommendations are based on adult data considering high-risk NET as defined by European Neuroendocrine Tumour Society (ENETS) guidelines for completion right-sided hemicolectomy (RHC). Recent data suggest that less aggressive therapy may be justified.
Analysis of children and adolescents with appNET prospectively registered with the German Malignant Endocrine Tumour (MET) studies between 1997 and 2022.
By December 2022, 662 patients (64.7% females, 35.3% male) had been reported. Median age was 13.3 years [4.5-17.9], median duration of follow-up 2.2 years [0-10.9]. No distant metastases were reported. Tumour size was <1 cm in 63.5%, 1-2 cm in 33.2%, and >2 cm in 3.2% of patients. WHO grade 1 and 2 tumours were diagnosed in 76.9% and 23.1% of patients, respectively. Lymphovascular invasion and lymph node metastases were associated with tumour size ≥1.5 cm. 27.0% of patients presented with high-risk NET according to ENETS criteria. Of those, only 55.9% underwent secondary oncological right hemicolectomy. Neither distant metastases, nor recurrences or disease-related deaths occurred in patients with appendectomy only as well as in patients with completion RHC. Overall and event-free survival were both 100%.
Internationally harmonized consensus recommendations on treatment of children and adolescents with appendiceal NET are urgently needed to avoid completion RHC in high-risk patients.
儿科阑尾神经内分泌肿瘤（appNET）非常罕见，主要通过阑尾切除术后的组织病理学评估偶然发现。治疗建议基于成人数据，考虑到欧洲神经内分泌肿瘤学会（ENETS）指南定义的高危神经内分泌肿瘤，需要完成右侧半结肠切除术（RHC）。最近的数据表明，较少的侵袭性治疗可能是合理的。
对 1997 年至 2022 年期间在德国恶性内分泌肿瘤（MET）研究中前瞻性登记的儿童和青少年 appNET 患者进行分析。
截至 2022 年 12 月，共报告了 662 例患者（女性占 64.7%，男性占 35.3%）。中位年龄为 13.3 岁[4.5-17.9]，中位随访时间为 2.2 年[0-10.9]。无远处转移报告。肿瘤大小<1cm 者占 63.5%，1-2cm 者占 33.2%，>2cm 者占 3.2%。WHO 分级 1 级和 2 级肿瘤分别占 76.9%和 23.1%。血管淋巴管侵犯和淋巴结转移与肿瘤大小≥1.5cm 相关。根据 ENETS 标准，27.0%的患者为高危神经内分泌肿瘤。其中，仅 55.9%的患者接受了二级肿瘤性右侧半结肠切除术。仅行阑尾切除术和完成 RHC 的患者均无远处转移、复发或疾病相关死亡。总生存和无事件生存均为 100%。
迫切需要制定国际统一的共识治疗建议，以避免对高危患者行 RHC。
