Prognosis after surgery for multiple endocrine neoplasia type 1-related pancreatic neuroendocrine tumors: Functionality matters.

Prognosis after surgery for multiple endocrine neoplasia type 1-related pancreatic neuroendocrine tumors: Functionality matters.
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DOI:
10.1016/j.surg.2020.09.037
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发表时间:
2021-04
期刊:
影响因子:
3.8
通讯作者:
Vriens, Menno R.
Vriens, Menno R.
中科院分区:
医学2区
文献类型:
--
作者:
van Beek, Dirk-Jan;Nell, Sjoerd;Verkooijen, Helena M.;Rinkes, Inne H. M. Borel;Valk, Gerlof D.;Vriens, Menno R.

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转移性胰腺神经内分泌肿瘤是1型多发性内分泌肿瘤患者死亡的主要原因。除了肿瘤大小,胰腺神经内分泌肿瘤的预后因素在很大程度上是未知的。本研究旨在评估多发性内分泌瘤1型相关的无功能胰腺神经内分泌肿瘤切除患者的预后是否与多发性内分泌瘤1型相关的胰岛素瘤切除患者不同,并评估与预后相关的因素。在1990年至2016年期间接受多发性内分泌瘤1型相关胰腺神经内分泌肿瘤切除术的患者在2个数据库中确定:荷兰MEN研究组和国际MEN 1胰岛素瘤研究组数据库。采用考克斯回归分析比较无功能性胰腺神经内分泌肿瘤患者与胰岛素瘤患者的无肝转移生存率,并确定与无肝转移生存率相关的因素。在153例多发性内分泌瘤1型患者中,61例因无功能性胰腺神经内分泌瘤而接受切除术,92例因胰岛素瘤而接受切除术。在淋巴结切除的患者中,56%(18/32)的无功能胰腺神经内分泌肿瘤有淋巴结转移,而胰岛素瘤有10%(4/41)(P = .001)。无功能性胰腺神经内分泌肿瘤的10年无肝癌生存率估计为63%(95%置信区间42%-76%),胰岛素瘤为87%(72%-91%)。在调整大小、世界卫生组织肿瘤分级和年龄后,无功能性胰腺神经内分泌肿瘤肝转移或死亡的风险增加(风险比3.04 [1.47-6.30])。在≥2 cm的胰腺神经内分泌肿瘤中,无功能性胰腺神经内分泌肿瘤(2.99 [1.22-7.33])和世界卫生组织2级(2.95 [1.02-8.50])与无肝癌生存率相关。与胰岛素瘤患者相比,多发性内分泌瘤1型相关的无功能性胰腺神经内分泌肿瘤患者的无肝癌生存率显著降低。术后咨询和随访方案应针对肿瘤类型,至少考虑肿瘤大小和世界卫生组织分级。
Metastasized pancreatic neuroendocrine tumors are the leading cause of death in patients with multiple endocrine neoplasia type 1. Aside from tumor size, prognostic factors of pancreatic neuroendocrine tumors are largely unknown. The present study aimed to assess whether the prognosis of patients with resected multiple endocrine neoplasia type 1-related nonfunctioning pancreatic neuroendocrine tumors differs from those with resected multiple endocrine neoplasia type 1-related insulinomas and assessed factors associated with prognosis. Patients who underwent resection of a multiple endocrine neoplasia type 1-related pancreatic neuroendocrine tumors between 1990 and 2016 were identified in 2 databases: the DutchMEN Study Group and the International MEN1 Insulinoma Study Group databases. Cox regression was performed to compare liver metastases-free survival of patients with a nonfunctioning pancreatic neuroendocrine tumors versus those with an insulinoma and to identify factors associated with liver metastases-free survival. Out of 153 patients with multiple endocrine neoplasia type 1, 61 underwent resection for a nonfunctioning pancreatic neuroendocrine tumor and 92 for an insulinoma. Of the patients with resected lymph nodes, 56% (18/32) of nonfunctioning pancreatic neuroendocrine tumors had lymph node metastases compared to 10% (4/41) of insulinomas (P = .001). Estimated 10-year liver metastases-free survival was 63% (95% confidence interval 42%–76%) for nonfunctioning pancreatic neuroendocrine tumors and 87% (72%–91%) for insulinomas. After adjustment for size, World Health Organization tumor grade, and age, nonfunctioning pancreatic neuroendocrine tumors had an increased risk for liver metastases or death (hazard ratio 3.04 [1.47–6.30]). In pancreatic neuroendocrine tumors ≥2 cm, nonfunctioning pancreatic neuroendocrine tumors (2.99 [1.22–7.33]) and World Health Organization grade 2 (2.95 [1.02–8.50]) were associated with liver metastases-free survival. Patients with resected multiple endocrine neoplasia type 1-related nonfunctioning pancreatic neuroendocrine tumors had a significantly lower liver metastases-free survival than patients with insulinomas. Postoperative counseling and follow-up regimens should be tumor type specific and at least consider size and World Health Organization grade.
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