Diagnosis and Treatment of Gastroenteropancreatic Neuroendocrine Tumors: Current Data on a Prospectively Collected, Retrospectively Analyzed Clinical Multicenter Investigation

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 Austri...

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Bibliographic Details
Published in:The oncologist (Dayton, Ohio) Ohio), 2011-01, Vol.16 (5), p.602-613
Main Authors: Niederle, Martin B., Niederle, Bruno
Format: Article
Language:English
Subjects:
Austria - epidemiology
Colonic Neoplasms - diagnosis
Colonic Neoplasms - epidemiology
Diagnosis and treatment
Disease Progression
Endocrinology
Gastrointestinal tract
Humans
Ileal Neoplasms - diagnosis
Ileal Neoplasms - epidemiology
Ileal Neoplasms - therapy
Intestinal Neoplasms - diagnosis
Intestinal Neoplasms - epidemiology
Intestinal Neoplasms - therapy
Jejunal Neoplasms - diagnosis
Jejunal Neoplasms - epidemiology
Jejunal Neoplasms - therapy
Multicenter Studies as Topic
Neoplasm Metastasis
Neoplasm Staging
Neuroendocrine tumors
Neuroendocrine Tumors - diagnosis
Neuroendocrine Tumors - epidemiology
Neuroendocrine Tumors - therapy
Pancreatic Neoplasms - diagnosis
Pancreatic Neoplasms - epidemiology
Pancreatic Neoplasms - therapy
Prospective clinical trial
Prospective Studies
Rectal Neoplasms - diagnosis
Rectal Neoplasms - epidemiology
Rectal Neoplasms - therapy
Retrospective Studies
Stomach Neoplasms - diagnosis
Stomach Neoplasms - epidemiology
Stomach Neoplasms - therapy
Surveys and Questionnaires
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Summary: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. 摘要 背景. 本研究采用前瞻性收集并回顾性分析的方法，旨在观察奥地利一年内确诊的胃肠胰腺神经内分泌瘤（GEP‐NETs）的“隐匿性”临床症状及诊断方法、治疗手段与短期随访措施。 方法. 本研究共纳入277例GEP‐NETs确诊患者。所有肿瘤均经最新免疫组化诊断标准（世界卫生组织、欧洲神经内分泌肿瘤协会）确诊。主治医师需填写一份包括50个临床与生化参数（临床症状、诊断方式、治疗、随访）的标准化问卷。 结果. 患者最常见的初发症状多为腹痛发作、腹泻、体重减轻、胃肠道出血、面色潮红及肠梗阻。总体而言，48.1%的肿瘤经内镜确诊、43.7%在手术中确诊、5%由原发瘤或转移瘤的细针穿刺检查确诊、2.5%在尸检时确诊；44.5%的肿瘤临床上未疑诊、在各种手术期间偶然确诊。总体而言，18.7%的肿瘤经内镜切除、67.6%由手术切除；另13.7%的患者仅随访、未接受干预性治疗。内镜或手术干预占81.4%，姑息性干预占18.6%。确诊时，83.7%的恶性NETs患者有转移方面的信息。淋巴结或远处转移占74.7%。19.3%的患者中记载41种继发性肿瘤，其中78.0%组织学分类为腺癌。 结论. 本研究总结GEP‐NETs的临床表现及治疗现状，由此拓展疾病认知和临床经验。 Clinical information concerning diagnosis, symptoms, a
ISSN:1083-7159
1549-490X
DOI:10.1634/theoncologist.2011-0002