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类癌综合征对胃肠胰神经内分泌肿瘤患者手术切除的临床特征和结局的影响。

Influence of carcinoid syndrome on the clinical characteristics and outcomes of patients with gastroenteropancreatic neuroendocrine tumors undergoing operative resection.

机构信息

Division of Surgical Oncology, The Ohio State University Comprehensive Cancer Center, Columbus, OH.

Division of Surgical Oncology, Department of Surgery, Winship Cancer Institute, Emory University, Atlanta, GA.

出版信息

Surgery. 2019 Mar;165(3):657-663. doi: 10.1016/j.surg.2018.09.008. Epub 2018 Oct 28.

Abstract

BACKGROUND

The incidence, clinical characteristics, and long-term outcomes of patients with gastroenteropancreatic neuroendrocrine tumors and carcinoid syndrome undergoing operative resection have not been well characterized.

METHODS

Patients undergoing resection of primary or metastatic gastroenteropancreatic neuroendrocrine tumors between 2000 and 2016 were identified from an 8-institution collaborative database. Clinicopathologic and postoperative characteristics as well as overall survival and disease-free survival were compared among patients with and without carcinoid syndrome.

RESULTS

Among 2,182 patients who underwent resection, 139 (6.4%) had preoperative carcinoid syndrome. Patients with carcinoid syndrome were more likely to have midgut primary tumors (44.6% vs 21.4%, P < .001), lymph node metastasis (63.4% vs 44.3%, P < .001), and metastatic disease (62.8% vs 26.7%, P < .001). There was no difference in tumor differentiation, grade, or Ki67 status. Perioperative carcinoid crisis was rare (1.6% vs 0%, P < .01), and the presence of preoperative carcinoid syndrome was not associated with postoperative morbidity (38.8% vs 45.5%, P = .129). Substantial symptom improvement was reported in 59.5% of patients who underwent curative-intent resection, but occurred in only 22.7% who underwent debulking. Despite an association on univariate analysis (P = .04), carcinoid syndrome was not independently associated with disease-free survival after controlling for confounding factors (hazard ratio 0.97, 95% confidence interval 0.64-1.45). Preoperative carcinoid syndrome was not associated with overall survival on univariate or multivariate analysis.

CONCLUSION

Among patients undergoing operative resection of gastroenteropancreatic neuroendrocrine tumors, the prevalence of preoperative carcinoid syndrome was low. Although operative intervention with resection or especially debulking in patients with carcinoid syndrome was disappointing and often failed to improve symptoms, after controlling for markers of tumor burden, carcinoid syndrome was not independently associated with worse disease-free survival or overall survival.

摘要

背景

患有胃肠胰神经内分泌肿瘤和类癌综合征并接受手术切除的患者的发病率、临床特征和长期预后尚未得到很好的描述。

方法

从 8 个机构合作的数据库中确定了 2000 年至 2016 年间接受原发性或转移性胃肠胰神经内分泌肿瘤切除术的患者。比较了有和没有类癌综合征的患者的临床病理和术后特征以及总生存率和无病生存率。

结果

在接受手术切除的 2182 名患者中,有 139 名(6.4%)患者术前患有类癌综合征。患有类癌综合征的患者更有可能患有中肠原发性肿瘤(44.6%比 21.4%,P<0.001)、淋巴结转移(63.4%比 44.3%,P<0.001)和转移性疾病(62.8%比 26.7%,P<0.001)。肿瘤分化、分级或 Ki67 状态无差异。围手术期类癌危象罕见(1.6%比 0%,P<0.01),术前类癌综合征的存在与术后发病率无关(38.8%比 45.5%,P=0.129)。接受根治性切除术的患者中有 59.5%报告了明显的症状改善,但接受减瘤术的患者中只有 22.7%报告了明显的症状改善。尽管单因素分析显示存在关联(P=0.04),但在控制混杂因素后,类癌综合征与无病生存率无关(风险比 0.97,95%置信区间 0.64-1.45)。单因素和多因素分析均显示术前类癌综合征与总生存率无关。

结论

在接受胃肠胰神经内分泌肿瘤手术切除的患者中,术前类癌综合征的患病率较低。尽管在类癌综合征患者中进行手术干预,包括切除术或特别是减瘤术,效果令人失望,往往无法改善症状,但在控制肿瘤负荷标志物后,类癌综合征与无病生存率或总生存率无关。

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