1型多发性内分泌腺瘤病患者神经内分泌肿瘤的转移灶切除术

Metastasectomy of neuroendocrine tumors in patients with multiple endocrine neoplasia type 1.

作者信息

Dy Benzon M, Que Florencia G, Thompson Geoffrey B, Young William F, Rowse Phillip, Strajina Veljko, Richards Melanie L

机构信息

Department of Surgery, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.

Department of Endocrinology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.

出版信息

Am J Surg. 2014 Dec;208(6):1047-53; discussion 1052-3. doi: 10.1016/j.amjsurg.2014.08.010. Epub 2014 Sep 22.

DOI:10.1016/j.amjsurg.2014.08.010

PMID:25440487

Abstract

BACKGROUND

Neuroendocrine (NE) tumors commonly afflict patients with multiple endocrine neoplasia type 1 (MEN1). It is thought that patients with MEN1 have improved survival compared with individuals with analogous lesions. The role of metastasectomy of NE tumors in MEN1 patients is not clearly defined.

METHODS

A review of MEN1 patients undergoing surgery for NE tumors from 1994 to 2010 at a single tertiary care center was performed. Tumor function, the extent of metastasis, R0 resection, and survival were analyzed.

RESULTS

We identified 30 patients who underwent resection including synchronous and metachronous metastasectomy. Synchronous metastases were identified in 19 patients (63%), whereas 11 (37%) had metachronous disease. R0 resection was achieved in 93% of patients. Estimated 10-year survival is 86.4% (95% confidence interval, 60% to 100%) with no factors predictive of overall survival. The disease-free interval at 1, 5, and 10 years was 89%, 50%, and 19%, respectively, with recurrence occurring at a median of 5.4 years (95% confidence interval, 77.7% to 100%). Synchronous metastasis (P = .0072; hazard ratio [HR], 3.4) and nonfunctioning tumors (P = .014; HR, 3.3) were more likely to recur, whereas age (P = .09; HR, 1.5), gender (P = .49; HR, 1.3), and the site of metastasis (P = .81; HR, 1.1) did not influence recurrence.

DISCUSSION

Patients with MEN1 benefit from resection of metastatic NE disease. Despite a high recurrence rate, survival and disease-free interval is favorable vs patients without MEN1.

摘要

背景

神经内分泌（NE）肿瘤常见于1型多发性内分泌腺瘤病（MEN1）患者。据认为，与患有类似病变的个体相比，MEN1患者的生存率有所提高。NE肿瘤转移灶切除术在MEN1患者中的作用尚未明确界定。

方法

对1994年至2010年在一家三级医疗中心接受NE肿瘤手术的MEN1患者进行回顾性研究。分析肿瘤功能、转移范围、R0切除情况和生存率。

结果

我们确定了30例接受切除术的患者，包括同期和异时转移灶切除术。19例患者（63%）发现有同期转移，而11例（37%）有异时性疾病。93%的患者实现了R0切除。估计10年生存率为86.4%（95%置信区间，60%至100%），且无总体生存的预测因素。1年、5年和10年的无病间期分别为89%、50%和19%，复发的中位时间为5.4年（95%置信区间，77.7%至100%）。同期转移（P = .0072；风险比[HR]，3.4）和无功能肿瘤（P = .014；HR，3.3）更易复发，而年龄（P = .09；HR，1.5）、性别（P = .49；HR，1.3）和转移部位（P = .81；HR，1.1）不影响复发。