Mainetti Martina, Aprile Maria Rosaria, Campana Davide, Marchetti Federico
Uoc Pediatria e neonatologia, Ospedale di Ravenna, Ausl della Romagna.
Dipartimento di Anatomia patologica, Ospedale di Ravenna, Ausl Romagna.
Recenti Prog Med. 2024 Jul-Aug;115(7):361-365. doi: 10.1701/4314.42991.
Well-differentiated neuroendocrine tumors of the appendix (NETs) are rare in pediatric and adolescent age groups. However, they are the most common gastrointestinal epithelial tumor in this age group and the most common malignancy of the appendix in the general population. The classification of these tumors considers factors such as the proliferation index, size of the neoplasm, and the presence of perineural and/or lymphovascular invasion, which can contribute to distant metastases. Preoperative diagnosis is challenging, except in cases where patients exhibit symptoms of carcinoid syndrome or signs of metastatic disease, which are uncommon in pediatric and adolescent patients. For tumors smaller than 1 cm, appendectomy is usually curative, while larger tumors or those at risk of spreading may require right hemicolectomy with lymphadenectomy. We present a case of an adolescent with NET and provide a literature review on the diagnostic and therapeutic approaches that should be considered for this relatively rare condition.Key words. Adolescent age, appendix, neuroendocrine tumors, pediatric age.
阑尾高分化神经内分泌肿瘤（NETs）在儿童和青少年年龄组中较为罕见。然而，它们是该年龄组中最常见的胃肠道上皮肿瘤，也是一般人群中最常见的阑尾恶性肿瘤。这些肿瘤的分类考虑增殖指数、肿瘤大小以及神经周围和/或淋巴管侵犯等因素，这些因素可能导致远处转移。术前诊断具有挑战性，除非患者表现出类癌综合征症状或转移性疾病体征，而这些在儿童和青少年患者中并不常见。对于小于1厘米的肿瘤，阑尾切除术通常可治愈，而较大的肿瘤或有扩散风险的肿瘤可能需要行右半结肠切除术并进行淋巴结清扫。我们报告一例青少年NET病例，并对这种相对罕见疾病应考虑的诊断和治疗方法进行文献综述。关键词：青少年年龄、阑尾、神经内分泌肿瘤、儿童年龄。
