Department of General Surgery, Qilu Hospital, Shandong University, Jinan, Shandong Province, 250012, China.
Department of Pulmonary and Critical Care Medicine, Qilu Hospital Shandong University, Jinan, 250012, China.
Pancreatology. 2019 Jul;19(5):738-750. doi: 10.1016/j.pan.2019.05.462. Epub 2019 May 27.
BACKGROUND/OBJECTIVES: The clinicopathological features and biological behaviors of cystic pancreatic neuroendocrine tumors (pNETs) are unclear and controversial. Here we performed a systematic review and meta-analysis to investigate the unique characteristics of cystic pNETs, to determine whether they represent a distinct clinical entity.
We selected comparative studies published since January 2000 that explore the differences between clinicopathological features of cystic and solid pNETs. Demographic information, pathological characteristics, and survival information were analyzed.
The 12 selected studies comprised 355 and 1530 patients diagnosed with cystic and solid pNETs, respectively. Compared with solid pNETs, cystic pNETs were less likely to be functional (odds ratio, OR = 0.31, 95% confidence interval (CI) 0.19-0.50, p < 0.00001), more likely to affect males (OR = 1.56, 95% CI 1.22-2.00, p = 0.0005), and significantly associated with multiple endocrine neoplasia type 1 (OR = 2.71). Cystic pNETs were more likely to present with G1 and G2 rather than G3 (OR = 1.66). Cystic pNETs were associated with less frequent distant organs and lymph node metastasis, microvascular invasion, perineural invasion, and a low Ki-67 index and mitotic count. There were no significant differences between 5- and 10-year overall survival. However, the 5-year disease-free survival (DFS) and 10-year DFS rate of patients with cystic pNETs was significantly higher compared with those with solid pNETs (94.6% vs 83.5%, OR = 3.00; 92.7% vs 63.6%, OR = 5.92, respectively).
Cystic pNETs represent a distinct subgroup of pNETs that present with an indolent biological behavior, and patients experience better DFS. Observation and surveillance should be considered in some selected cases.
背景/目的：囊性胰腺神经内分泌肿瘤（pNET）的临床病理特征和生物学行为尚不清楚且存在争议。本研究通过系统回顾和荟萃分析，旨在探讨囊性 pNET 的独特特征，以确定其是否代表一种独特的临床实体。
我们选择了自 2000 年 1 月以来发表的比较研究，以探讨囊性和实性 pNET 之间的临床病理特征差异。分析了人口统计学信息、病理特征和生存信息。
12 项入选研究共纳入 355 例囊性和 1530 例实性 pNET 患者。与实性 pNET 相比，囊性 pNET 发生功能性肿瘤的可能性更小（比值比，OR=0.31，95%置信区间[CI] 0.19-0.50，p<0.00001），更可能影响男性（OR=1.56，95%CI 1.22-2.00，p=0.0005），且与多发性内分泌肿瘤 1 型显著相关（OR=2.71）。囊性 pNET 更可能表现为 G1 和 G2，而非 G3（OR=1.66）。囊性 pNET 与远处器官和淋巴结转移、微血管侵犯、神经周围侵犯以及低 Ki-67 指数和有丝分裂计数的发生率较低相关。5 年和 10 年总生存率无显著差异。然而，囊性 pNET 患者的 5 年无疾病生存率（DFS）和 10 年 DFS 率显著高于实性 pNET 患者（94.6%比 83.5%，OR=3.00；92.7%比 63.6%，OR=5.92）。
囊性 pNET 是 pNET 的一个独特亚组，其生物学行为呈惰性，患者 DFS 更好。在某些选定的病例中，应考虑观察和监测。
