Gastroenterology and Endoscopy Unit, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Università degli Studi di Milano, Milan, Italy.
Department of Medical and Surgical Sciences, Bologna University St. Orsola-Malpighi Polyclinic Hospital, Bologna, Italy.
Ann Surg Oncol. 2018 Oct;25(11):3200-3206. doi: 10.1245/s10434-018-6673-5. Epub 2018 Jul 27.
The optimal management of duodenal neuroendocrine neoplasms (dNENs) is unclear, and endoscopic resection is increasingly performed instead of surgery.
This is a retrospective analysis of patients with histologically confirmed diagnosis of dNENs, managed at five Italian tertiary referral Centers in Italy.
From 2000 to 2017, 108 patients (69 males, 39 females, median age 59.5 years) were included in this study. Seventy-one patients had G1, 21 G2, 4 G3 dNENs (12 Ki-67 not available). Fifty-four patients showed metastases at diagnosis, and 20 patients developed metachronous metastases. Thirty patients had a functioning dNEN (14 metastatic). Fifty-seven patients had the dNEN surgically resected, 16 endoscopically, 23 metastatic, received medical therapy + surgery or endoscopy. Seven patients underwent liver-directed therapies, and one patient had PRRT. Median OS was 187 months. During a median follow-up of 76 months, 20 patients died (19 of disease-related causes). At Cox's multivariate proportional hazard regression, grading and age were the only variables independently related to OS. Median PFS was 170 months. Grading and staging at the initial diagnosis were independently related to PFS. No differences in terms of OS and PFS were observed between patients treated surgically or endoscopically.
dNENs prognosis may be highly variable. These tumors can be metastatic in up to 50% of cases at the time of first diagnosis and can develop metastases thereafter. Functioning neoplasms express high metastatic potential. Nuclear imaging should be performed to exclude distant metastases in all dNENs. Endoscopy and surgery play a primary role in the management of the disease. Further prospective studies are needed.
十二指肠神经内分泌肿瘤（dNENs）的最佳治疗方法尚不清楚，内镜下切除的应用日益增多，而手术切除的应用则逐渐减少。
这是对意大利五家三级转诊中心确诊为 dNENs 的患者进行的回顾性分析。
2000 年至 2017 年，共有 108 例患者（69 例男性，39 例女性，中位年龄 59.5 岁）纳入本研究。71 例患者为 G1 级，21 例患者为 G2 级，4 例患者为 G3 级（12 例 Ki-67 未提供）。54 例患者在诊断时即发生转移，20 例患者发生异时性转移。30 例患者为功能性 dNEN（14 例发生转移）。57 例患者接受了 dNEN 手术切除，16 例患者接受了内镜下切除，23 例患者接受了转移性疾病的药物治疗+手术或内镜治疗。7 例患者接受了肝定向治疗，1 例患者接受了 PRRT。中位总生存期为 187 个月。在中位随访 76 个月期间，20 例患者死亡（19 例死于疾病相关原因）。在 Cox 多变量比例风险回归分析中，分级和年龄是唯一与总生存期相关的变量。中位无进展生存期为 170 个月。初诊时的分级和分期与无进展生存期独立相关。手术或内镜治疗的患者在总生存期和无进展生存期方面无差异。
dNENs 的预后可能差异很大。这些肿瘤在首次诊断时多达 50%的病例可能发生转移，此后可能会发生转移。功能性肿瘤具有较高的转移潜能。所有 dNENs 都应进行核素成像以排除远处转移。内镜和手术在疾病的治疗中发挥主要作用。需要进一步开展前瞻性研究。
