Schwerpunkt Endokrinologie und Stoffwechselerkrankungen, I. Medizinischen Klinik und Poliklinik; ENETS center of excellence, Universitätsmedizin der Johannes Gutenberg-Universität Mainz, Langenbeckstrasse 1, 55101, Mainz, Germany.
Department of Medicine-DIMED, University of Padova, Padova, Italy.
Rev Endocr Metab Disord. 2017 Dec;18(4):393-410. doi: 10.1007/s11154-017-9438-8.
Neuroendocrine neoplasms of the digestive system (GEP-NEN) represent a heterogeneous group of malignancies with various clinical presentation and prognosis. GEP-NENs can potentially affect all organs of the gastrointestinal tract; characteristically they share the biological property to produce and secrete peptides and neuroamines. About 30% of GEP-NENs are hormonally active and can cause specific clinical syndromes. The clinical presentation mainly depends on the primary site of the tumor and its functionality. Because of the wide spectrum of clinical symptoms and their misperceived rarity, diagnosis of GEP-NENs is often delayed for years and tumors are detected first in an advanced stage. Early identification of a specific hormonal syndrome can significantly impact tumor diagnosis and treatment, moreover the preoperative management of NEN hormonal release avoids potential life threatening hormonal crisis. However, GEP-NEN diagnostic work-up is challenging, it requires a multidisciplinary team and needs particular experience; standardized protocols and clinical experience are essential for a proper endocrine diagnostic work-up. In addition to the biochemical diagnostic, further radiologic and endoscopic imaging modalities are required moreover, somatostatin-receptor based functional imaging, using either Octreotide-scintigraphy or novel PET-based techniques with specific isotopes like Ga-DOTA-octreotate, plays an important role for the detection of the primary tumor as well as for the evaluation of the tumor extent.
消化系统神经内分泌肿瘤（GEP-NEN）是一组具有不同临床表现和预后的恶性肿瘤，具有异质性。GEP-NEN 可能会影响胃肠道的所有器官；其特征是具有产生和分泌肽类和神经胺的生物学特性。约 30%的 GEP-NEN 具有激素活性，并可能导致特定的临床综合征。临床表现主要取决于肿瘤的原发部位及其功能。由于临床症状广泛且被误认为罕见，因此 GEP-NEN 的诊断通常会延迟数年，而且肿瘤通常在晚期才被发现。特定激素综合征的早期识别可显著影响肿瘤的诊断和治疗，此外，NEN 激素释放的术前管理可避免潜在的威胁生命的激素危机。然而，GEP-NEN 的诊断工作具有挑战性，需要多学科团队的参与，并且需要特定的经验；标准化的方案和临床经验对于适当的内分泌诊断工作至关重要。除了生化诊断外，还需要进一步的放射学和内镜影像学检查，此外，基于生长抑素受体的功能成像，使用奥曲肽闪烁扫描或新型基于 PET 的技术，结合特定的同位素如 Ga-DOTA-奥曲肽，对于检测原发肿瘤以及评估肿瘤范围具有重要作用。
