胃肠胰神经内分泌肿瘤的诊断和治疗：前瞻性采集、回顾性分析的临床多中心研究的最新数据。
Diagnosis and treatment of gastroenteropancreatic neuroendocrine tumors: current data on a prospectively collected, retrospectively analyzed clinical multicenter investigation.
机构信息
Section of Endocrine Surgery, Division of General Surgery, Department of Surgery, Medical University of Vienna, Währinger Gür, Austria.
出版信息
Oncologist. 2011;16(5):602-13. doi: 10.1634/theoncologist.2011-0002. Epub 2011 Apr 5.
BACKGROUND
The aim of this prospectively collected, retrospectively analyzed clinical investigation was to describe "unmasked" clinical symptoms and methods of diagnosis, treatment, and short-term follow-up of gastroenteropancreatic neuroendocrine tumors (GEP-NETs) diagnosed during 1 year in Austria.
METHODS
In total, 277 patients with GEP-NETs were documented. All tumors were immunhistochemically defined according to recently summarized criteria (World Health Organization, European Neuroendocrine Tumour Society). A standardized questionnaire comprising 50 clinical and biochemical parameters (clinical symptoms, mode of diagnosis, treatment, follow-up) was completed by attending physicians.
RESULTS
The most common initial symptoms were episodes of abdominal pain, diarrhea, weight loss, gastrointestinal bleeding, flushing, and bowel obstruction. Overall, 48.1% of tumors were diagnosed by endoscopy, 43.7% were diagnosed during surgery, 5% were diagnosed by fine-needle aspiration of the primary or metastases, and 2.5% were diagnosed during autopsy; 44.5% of tumors were not suspected clinically and were diagnosed incidentally during various surgical procedures. Overall, 18.7% of tumors were removed endoscopically and 67.6% were removed surgically; 13.7% of patients were followed without interventional treatment. Endoscopic or surgical intervention was curative in 81.4% of patients and palliative in 18.6% of patients. At the time of diagnosis, information on metastasis was available in 83.7% of patients with malignant NETs. Lymph node or distant metastases were documented in 74.7% of patients. In 19.3% of patients, 41 secondary tumors were documented, with 78.0% classified histologically as adenocarcinomas.
CONCLUSION
This investigation summarizes the clinical presentation and current practice of management of GEP-NETs and thereby extends the understanding and clinical experience.
背景
本前瞻性收集、回顾性分析的临床研究旨在描述奥地利 1 年内诊断的胃肠胰神经内分泌肿瘤（GEP-NETs）的“未伪装”临床症状以及诊断、治疗和短期随访方法。
方法
共记录了 277 例 GEP-NET 患者。所有肿瘤均根据最近总结的标准（世界卫生组织、欧洲神经内分泌肿瘤学会）进行免疫组织化学定义。主治医生填写了一份包含 50 个临床和生化参数（临床症状、诊断方式、治疗、随访）的标准化问卷。
结果
最初最常见的症状是腹痛、腹泻、体重减轻、胃肠道出血、潮红和肠梗阻。总体而言，48.1%的肿瘤通过内镜诊断，43.7%通过手术诊断，5%通过原发或转移灶的细针抽吸诊断，2.5%通过尸检诊断；44.5%的肿瘤临床未被怀疑，在各种手术过程中意外诊断。总体而言，18.7%的肿瘤通过内镜切除，67.6%通过手术切除；13.7%的患者未经介入治疗进行随访。内镜或手术干预在 81.4%的恶性 NET 患者中是治愈性的，在 18.6%的患者中是姑息性的。在诊断时，有恶性 NET 患者 83.7%的患者有转移信息。74.7%的患者记录有淋巴结或远处转移。在 19.3%的患者中，记录了 41 个继发性肿瘤，其中 78.0%组织学上分类为腺癌。
结论
本研究总结了 GEP-NET 的临床表现和当前管理实践，从而扩展了对其的认识和临床经验。
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