Bolanowski Marek, Jarzab Barbara, Handkiewicz-Junak Daria, Jeziorski Arkadiusz, Kos-Kudła Beata, Zajecki Wojciech
Katedra i Klinika Endokrynologii, Diabetologii i Leczenia Izotopami, Akademia Medyczna, Wroclaw, Poland.
Endokrynol Pol. 2008 Jan-Feb;59(1):87-96.
Polish recommendations regarding management of patients suffering from neuroendocrine tumors of small intestine and appendix are presented. Small intestine, especially ileum represent most common origin of these tumors. Majority of them are well differentiated and grow slowly. Rarely, they are less differentiated with fast growth and poor prognosis. Symptoms are atypical, diagnosis could be often accidental. In 4-10% of patients typical symptoms of carcinoid syndrome are present. Chromogranin A is useful in the laboratory diagnostics, and urinary excretion of 5-hydroxyindoloacetic acid is helpful for the diagnostics and monitoring of the disease. Histopathological diagnostics was extensively described. Ultrasound, colonoscopy, capsule endoscopy, baloon enteroscopy, computed tomography, magnetic resonance and somatostatin analogs scintigraphy could be used for the visualization. The treatment of choice in the neuroendocrine tumors of small intestine and appendix is radical or palliative surgery, if possible using endoscopy. Pharmacotherapy consists of biotherapy and chemotherapy. The crucial in biotherapy is somatostatin analogs application, possible in symptomatic treatment of hormonally functioning tumors. This is treatment of choice in carcinoid crisis. Interferon alfa could be applied because of the same indications as somatostatin analogs, except for carcinoid crisis. Chemotherapy is less successful in disseminated or locally advanced intestinal neuroendocrine tumors, so radioisotope therapy should be considered in each case of unresectable tumor.
本文介绍了波兰关于小肠和阑尾神经内分泌肿瘤患者管理的建议。小肠，尤其是回肠是这些肿瘤最常见的起源部位。它们大多分化良好，生长缓慢。很少有分化较差、生长迅速且预后不良的情况。症状不典型，诊断往往是偶然的。4% - 10%的患者会出现类癌综合征的典型症状。嗜铬粒蛋白A在实验室诊断中有用，5 - 羟吲哚乙酸的尿排泄量有助于疾病的诊断和监测。文中对组织病理学诊断进行了详细描述。超声、结肠镜检查、胶囊内镜、气囊小肠镜检查、计算机断层扫描、磁共振成像和生长抑素类似物闪烁扫描可用于可视化检查。小肠和阑尾神经内分泌肿瘤的首选治疗方法是根治性或姑息性手术，如有可能可采用内镜手术。药物治疗包括生物治疗和化疗。生物治疗的关键是应用生长抑素类似物，可用于有激素功能肿瘤的对症治疗。这是类癌危象的首选治疗方法。由于与生长抑素类似物有相同的适应证，除类癌危象外，可应用干扰素α。化疗在播散性或局部晚期肠道神经内分泌肿瘤中效果较差，因此对于不可切除的肿瘤，每种情况都应考虑放射性核素治疗。
