Ventin Marco, Arya Shahrzad, Cattaneo Giulia, Fernandez-Del Castillo Carlos, Wei Alice C, Zureikat Amer H, Alvikas Jurgis, Javed Ammar A, Campbell Brady A, Sekigami Yurie, Zheng Jian, Qadan Motaz, Lillemoe Keith D, He Jin, Ferrone Cristina R
Department of Surgery, Massachusetts General Hospital, Harvard Medical School, Boston, MA.
Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY.
Ann Surg. 2025 Jan 9. doi: 10.1097/SLA.0000000000006624.
To investigate the clinicopathological features and long-term outcomes of cystic and solid pancreatic neuroendocrine tumors (PanNETs).
PanNETs uncommonly present as cystic lesions. Whether cystic PanNETs represent a distinct clinical entity compared to solid PanNETs is controversial.
Clinicopathologic data of patients with resected PanNETs were collected from 4 high-volume centers between 2000-2019. Clinicopathological characteristics and outcomes of patients with cystic and solid PanNETs were compared based on a 3 cm tumor size cut-off using the Chi-squared test and Mann-Whitney U test. Survival estimates were calculated with the Kaplan-Meier method and log-rank test and multivariable analysis using a Cox proportional hazard model.
Of the 1727 patients undergoing pancreatectomy for PanNET, the median age was 58.1 years (IQR, 18.4), and 53.3% were male. Of these, 177 (10.3%) were cystic and 1550 (89.7%) solid. Cystic PanNETs were more prevalent in patients with hereditary syndromes, less frequently functional, and more often located in the body/tail of the pancreas. After the exclusion of patients with functional tumors, WHO G3 tumors, hereditary syndromes, neoadjuvant treatment, and metastatic stage, 145 cystic PanNETs were compared to 1059 solid PanNETs, and the median follow-up period of the cohort was 64 months. Cystic PanNETs demonstrated significantly fewer high-risk histopathological features, lymph node metastases (5.5% vs. 24.0%, P<0.001), and distant recurrence (4.1% vs. 14.4%; P<0.001). Among tumors ≤3 cm, cystic PanNETs had a low rate of lymph node metastases (3.9% vs. 17.8%; P<0.001), recurrence (3.1% vs. 8.4%; P=0.041), and low propensity to recur distantly. Cystic PanNETs had favorable long-term survival regardless of tumor size.
Cystic PanNETs have a more benign course than their solid counterparts and conservative management can be considered for EUS-FNA-proven cystic PanNETs ≤3 cm. Parenchyma and lymph-node sparing resections are warranted in patients with cystic PanNETs>3 cm. Patients with poor baseline performance status may forego cystic PanNET resection and not affect their overall survival.
探讨囊性和实性胰腺神经内分泌肿瘤（PanNETs）的临床病理特征及长期预后。
PanNETs很少表现为囊性病变。与实性PanNETs相比，囊性PanNETs是否代表一种独特的临床实体存在争议。
收集2000年至2019年期间4个大型中心接受PanNETs切除术患者的临床病理数据。采用卡方检验和曼-惠特尼U检验，比较肿瘤大小以3 cm为界的囊性和实性PanNETs患者的临床病理特征及预后。采用Kaplan-Meier法、对数秩检验和Cox比例风险模型进行多变量分析计算生存估计值。
在1727例行PanNETs胰腺切除术的患者中，中位年龄为58.1岁（IQR，18.4），男性占53.3%。其中，177例（10.3%）为囊性，1550例（89.7%）为实性。囊性PanNETs在遗传性综合征患者中更常见，功能障碍较少见，且更常位于胰腺体/尾部。排除功能性肿瘤、WHO G3肿瘤、遗传性综合征、新辅助治疗和转移期患者后，将145例囊性PanNETs与1059例实性PanNETs进行比较，队列的中位随访期为64个月。囊性PanNETs的高风险组织病理学特征、淋巴结转移（5.5%对24.0%，P<0.001）和远处复发（4.1%对14.4%；P<0.001）明显较少。在≤3 cm的肿瘤中，囊性PanNETs的淋巴结转移率（3.9%对17.8%；P<0.001）、复发率（3.1%对8.4%；P=0.041）较低，远处复发倾向也较低。无论肿瘤大小，囊性PanNETs均具有良好的长期生存率。
囊性PanNETs的病程比实性PanNETs更良性，对于经EUS-FNA证实的≤3 cm的囊性PanNETs可考虑保守治疗。对于>3 cm的囊性PanNETs患者，应进行保留实质和淋巴结的切除术。基线表现状态较差的患者可放弃囊性PanNETs切除术，且不影响其总生存期。
