Pregun István, Bodoky György, Rácz Károly, Tulassay Zsolt
Semmelweis Egyetem, Általános Orvostudományi Kar II. Belgyógyászati Klinika Budapest.
Orv Hetil. 2010 Nov 14;151(46):1885-94. doi: 10.1556/OH.2010.29000.
The authors review the most important clinical aspects of carcinoid tumors. Carcinoid tumors originating in neuroendocrine cells are rare, usually slowly-growing neoplasms, however, they may present as aggressive and rapidly progressing tumors. Epidemiologic data indicates that their prevalence is gradually increasing, which may be explained, at least in part, by the development and wider use of advanced diagnostic methods. A considerable proportion of patients with neuroendocrine tumors are symptom-free, whereas others may have carcinoid syndrome or symptoms of other endocrine syndromes. Early diagnosis may be established by the measurement of biochemical markers (serum chromogranin A, urinary 5-hydroxyindoleacetic acid) and advanced localization methods. A considerable number of patients are diagnosed at the late stages of the disease; in these cases surgical cure is not possible but surgical and/or interventional radiologic procedures which reduce tumoral mass should be still considered. The most effective drugs for symptomatic treatment of carcinoid tumors are somatostatin analogues; in addition to their beneficial effect on clinical symptoms they may stabilize tumor growth for many years and, less frequently, may produce tumor regression. The use of chemotherapeutic agents is considered in patients with aggressive, rapidly growing and advanced tumors; initial findings with temozolomide and thalidomide in clinical trials raise the possibility that these chemotherapeutic agents may prove to be new therapeutic options. Radioisotope-labeled peptide receptor therapy with 131 I-MIBG, 90 Y-DOTA-TOC or 177 Lu-DOTA-TOC may offer a highly effective option for patients with progressive and advanced stage of neuroendocrine tumors. Initial observations obtained in clinical trials with some tyrosine kinase inhibitors, antibodies against tyrosine kinases, and with inhibitors of mammalian target of rapamycin (mTOR) support the possibility that at least some of these new agents may have a role in future treatment options in patients with advanced neuroendocrine tumors.
作者回顾了类癌肿瘤最重要的临床方面。起源于神经内分泌细胞的类癌肿瘤罕见，通常为生长缓慢的肿瘤，然而，它们也可能表现为侵袭性强且进展迅速的肿瘤。流行病学数据表明其患病率正在逐渐上升，这至少部分可以通过先进诊断方法的发展和更广泛应用来解释。相当一部分神经内分泌肿瘤患者没有症状，而其他患者可能有类癌综合征或其他内分泌综合征的症状。早期诊断可通过检测生化标志物（血清嗜铬粒蛋白A、尿5-羟吲哚乙酸）和先进的定位方法来确立。相当多的患者在疾病晚期才被诊断出来；在这些情况下，手术治愈已不可能，但仍应考虑采取减少肿瘤体积的手术和/或介入放射学程序。治疗类癌肿瘤症状最有效的药物是生长抑素类似物；除了对临床症状有有益作用外，它们还可能使肿瘤生长稳定多年，较少情况下可使肿瘤缩小。对于侵袭性强、生长迅速且处于晚期的肿瘤患者，可考虑使用化疗药物；替莫唑胺和沙利度胺在临床试验中的初步结果表明，这些化疗药物可能成为新的治疗选择。用131I-MIBG、90Y-DOTA-TOC或177Lu-DOTA-TOC进行放射性同位素标记的肽受体治疗，可能为进展期和晚期神经内分泌肿瘤患者提供一种高效的选择。在一些酪氨酸激酶抑制剂、抗酪氨酸激酶抗体以及雷帕霉素靶蛋白（mTOR）抑制剂的临床试验中获得的初步观察结果支持这样一种可能性，即至少其中一些新药可能在晚期神经内分泌肿瘤患者的未来治疗选择中发挥作用。
