Bednarczuk Tomasz, Bolanowski Marek, Zemczak Anna, Bałdys-Waligórska Agata, Blicharz-Dorniak Jolanta, Boratyn-Nowicka Agnieszka, Borowska Małgorzata, Cichocki Andrzej, Ćwikła Jarosław B, Falconi Massimo, Foltyn Wanda, Handkiewicz-Junak Daria, Hubalewska-Dydejczyk Alicja, Jarząb Barbara, Junik Roman, Kajdaniuk Dariusz, Kamiński Grzegorz, Kolasińska-Ćwikła Agnieszka, Kowalska Aldona, Król Robert, Królicki Leszek, Kunikowska Jolanta, Kuśnierz Katarzyna, Lampe Paweł, Lange Dariusz, Lewczuk-Myślicka Anna, Lewiński Andrzej, Lipiński Michał, Londzin-Olesik Magdalena, Marek Bogdan, Nasierowska-Guttmejer Anna, Nowakowska-Duława Ewa, Pałucki Jakub, Pilch-Kowalczyk Joanna, Rosiek Violetta, Ruchała Marek, Siemińska Lucyna, Sowa-Staszczak Anna, Starzyńska Teresa, Steinhof-Radwańska Katarzyna, Strzelczyk Janusz, Sworczak Krzysztof, Syrenicz Anhelli, Szawłowski Andrzej, Szczepkowski Marek, Wachuła Ewa, Zajęcki Wojciech, Zgliczyński Wojciech, Kos-Kudła Beata
Endokrynol Pol. 2017;68(2):223-236. doi: 10.5603/EP.2017.0018.
This study presents the revised Polish guidelines regarding the management of patients suffering from neuroendocrine neoplasms (NENs) of the small intestine and appendix. The small intestine, especially the ileum, is the most common location for these neoplasms. Most are well differentiated and slow growing. Their symptoms may be atypical, which can result in delayed or accidental diagnosis. Appendicitis is usually the first manifestation of NEN in this location. Typical symptoms of carcinoid syndrome occur in approximately 20-30% of patients suffering from small intestinal NENs with distant metastases. The main cause of death in patients with carcinoid syndrome is carcinoid heart disease. The most useful laboratory test is the determination of chromogranin A, while concentration of 5-hydroxyindoleacetic acid is helpful in the diagnostics of carcinoid syndrome. For visualisation, ultrasound, computed tomography, magnetic resonance imaging, colonoscopy, video capsule endoscopy, double-balloon enteroscopy, and somatostatin receptor scintigraphy may be used. A detailed his-tological report is crucial for the proper diagnostics and therapy of NENs of the small intestine and appendix. The treatment of choice is surgical management, either radical or palliative. The pharmacological treatment of the hormonally active and non-active small intestinal NENs as well as NENs of the appendix is based on long-acting somatostatin analogues. In patients with generalised NENs of the small intestine in progress during the SSA treatment, with good expression of somatostatin receptors, the first-line treatment should be radio-isotope therapy, while targeted therapies, such as everolimus, should be considered afterwards. When the above therapies are exhausted, in certain cases chemotherapy may be considered.
本研究介绍了波兰修订后的关于小肠和阑尾神经内分泌肿瘤（NENs）患者管理的指南。小肠，尤其是回肠，是这些肿瘤最常见的发生部位。大多数肿瘤分化良好且生长缓慢。其症状可能不典型，这可能导致诊断延迟或偶然发现。阑尾炎通常是该部位NEN的首发表现。类癌综合征的典型症状约在20% - 30%发生远处转移的小肠NEN患者中出现。类癌综合征患者的主要死因是类癌心脏病。最有用的实验室检查是嗜铬粒蛋白A的测定，而5 - 羟吲哚乙酸的浓度有助于类癌综合征的诊断。为进行可视化检查，可使用超声、计算机断层扫描、磁共振成像、结肠镜检查、视频胶囊内镜检查、双气囊小肠镜检查和生长抑素受体闪烁显像。详细的组织学报告对于小肠和阑尾NEN的正确诊断和治疗至关重要。治疗的首选方法是手术管理，包括根治性或姑息性手术。小肠和阑尾的激素活性和非活性NEN的药物治疗基于长效生长抑素类似物。在长效生长抑素类似物（SSA）治疗期间进展为广泛性小肠NEN且生长抑素受体表达良好的患者中，一线治疗应为放射性同位素治疗，之后应考虑使用依维莫司等靶向治疗。当上述治疗方法均用尽时，在某些情况下可考虑化疗。
