Huang Yu-Chen, Yang Ning-Ning, Chen Hong-Chun, Huang Yuan-Li, Yan Wen-Tian, Yang Ru-Xue, Li Nan, Zhang Shan, Yang Pan-Pan, Feng Zhen-Zhong
Department of Pathology, The First Affiliated Hospital of Bengbu Medical College, Bengbu 233000, Anhui Province, China.
Department of Pathology, The First Affiliated Hospital of Anhui Medical University, Hefei 230000, Anhui Province, China.
World J Gastroenterol. 2021 Feb 21;27(7):624-640. doi: 10.3748/wjg.v27.i7.624.
The incidence of mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) is low. To improve our understanding of this rare tumor type and optimally guide clinical treatment, associated risk factors, clinical manifestations, and prognosis must be explored.
To identify risk factors that influence the prognosis of patients with gastroenteropancreatic MiNEN (GEP-MiNEN).
We retrospectively analyzed the clinical data of 46 patients who were diagnosed with GEP-MiNEN at the First Affiliated Hospital of Bengbu Medical College (Anhui, China) between January 2013 and December 2017. Risk factors influencing the prognosis of the patients were assessed using Kaplan-Meier curves and cox regression models. We compared the results with 55 randomly selected patients with gastroenteropancreatic GEP neuroendocrine tumors, 47 with neuroendocrine carcinomas (NEC), and 58 with poorly differentiated adenocarcinoma.
Among the 46 patients with GEP-MiNEN, thirty-five had gastric tumors, nine had intestinal tumors (four in the small intestine and five in the colon and rectum), and two had pancreatic tumors. The median age of the patients was 66 (41-84) years, and the male-to-female ratio was 2.83. Thirty-three (71.7%) patients had clinical stage III and IV cancers. Distant metastasis occurred in 14 patients, of which 13 had metastasis to the liver. The follow-up period was 11-72 mo, and the median overall survival was 30 mo. Ki-67 index ≥ 50%, high proportion of NEC, lymph node involvement, distant metastasis, and higher clinical stage were independent risk factors affecting the prognosis of patients with GEP-MiNEN. The median overall survival was shorter for patients with NEC than for those with MiNEN (14 mo 30 mo, = 0.001), but did not significantly differ from those with poorly differentiated adenocarcinoma and MiNEN (30 mo 18 mo, = 0.453).
A poor prognosis is associated with rare, aggressive GEP-MiNEN. Ki-67 index, tumor composition, lymph node involvement, distant metastasis, and clinical stage are important factors for patient prognosis.
混合性神经内分泌-非神经内分泌肿瘤（MiNEN）的发病率较低。为了增进我们对这种罕见肿瘤类型的了解并优化临床治疗指导，必须探索相关危险因素、临床表现和预后情况。
确定影响胃肠胰MiNEN（GEP-MiNEN）患者预后的危险因素。
我们回顾性分析了2013年1月至2017年12月期间在蚌埠医学院第一附属医院（中国安徽）被诊断为GEP-MiNEN的46例患者的临床资料。使用Kaplan-Meier曲线和Cox回归模型评估影响患者预后的危险因素。我们将结果与55例随机选择的胃肠胰GEP神经内分泌肿瘤患者、47例神经内分泌癌（NEC）患者和58例低分化腺癌患者进行了比较。
在46例GEP-MiNEN患者中，35例有胃部肿瘤，9例有肠道肿瘤（4例在小肠，5例在结肠和直肠），2例有胰腺肿瘤。患者的中位年龄为66（41-84）岁，男女比例为2.83。33例（71.7%）患者患有临床III期和IV期癌症。14例患者发生远处转移，其中13例转移至肝脏。随访时间为11-72个月，中位总生存期为30个月。Ki-67指数≥50%、NEC比例高、淋巴结受累、远处转移和更高的临床分期是影响GEP-MiNEN患者预后的独立危险因素。NEC患者的中位总生存期短于MiNEN患者（14个月对30个月，P = 0.001），但与低分化腺癌和MiNEN患者相比无显著差异（30个月对18个月，P = 0.453）。
罕见的侵袭性GEP-MiNEN预后不良。Ki-67指数、肿瘤组成、淋巴结受累、远处转移和临床分期是影响患者预后的重要因素。
