Sayeg Y, Sayeg M, Baum R P, Kulkarni H R, Presselt N, Mäder I, Kunze A, Sänger J, Hörsch D, Bonnet R
Klinik für Pneumologie der Zentralklinik Bad Berka GmbH.
Zentrum für Neuroendokrine Tumore Bad Berka - ENETS Center of Excellence und Klinik für Innere Medizin, Gastroenterologie und Endokrinologie.
Pneumologie. 2014 Jul;68(7):456-77. doi: 10.1055/s-0034-1365642. Epub 2014 Jul 9.
The pulmonary neuroendocrine neoplasms originate from the enterochromaffin cells which are diffusely distributed in the body. The incidence of these tumors has increased significantly in recent decades due to the available diagnostics. They make up about 1-2% of all lung tumors and 20-30% of all neuroendocrine neoplasms. The current WHO classification from 2004 divides them into typical carcinoids (TC), atypical carcinoids (AC), large cell neuroendocrine carcinomas (LCNEC) and small cell carcinomas (SCLC). The major neuroendocrine biomarkers are chromogranin A, synaptophysin and CD56. TC have a low mitotic rate of <2 mitoses/2mm(2) (10 HPF), whereas the mitotic rate of the AC is 2-10 mitoses/2 mm(2) (10 HPF). The Ki-67 staining is helpful to distinguish typical and atypical carcinoids from the highly malignant LCNEC and SCLC. Clinically, the patient presents usually with cough, hemoptysis or bronchial obstruction. The occurrence of a carcinoid or Cushing's syndrome and a tumor-associated acromegaly are rare. Surgical resection with radical lymph node dissection is the treatment of choice for achieving long-term survival. Endoscopic resection of the endobronchial tumor growth is a good alternative for inoperable endobronchially localized tumors. Peptide receptor radionuclide therapy (PRRT) is a promising treatment option for patients with metastatic or unresectable pulmonary neuroendocrine tumors. New targeted therapies using angiogenesis inhibitors, mTOR inhibitors, and tyrosine kinase inhibitors are being tested for their effectiveness in many previous studies. Typical carcinoid tumors metastasize less frequently than AC, the 5-year survival rate of patients with TC being over 90%. Patients with AC have a 5-year survival rate between 35% and 87%. The highly malignant LCNEC and SCLC, on the other hand, have a 5-year survival rate between 15% and 57%, and <5% respectively. The increasing number of therapeutic options and diagnostic procedures requires a multidisciplinary approach and decision-making in multidisciplinary tumor conferences to ensure a personalized treatment approach. Therefore patients with a neuroendocrine neoplasm of the lung should be treated in specialized centers.
肺神经内分泌肿瘤起源于广泛分布于体内的嗜银细胞。由于现有诊断方法的应用，近几十年来这些肿瘤的发病率显著增加。它们约占所有肺部肿瘤的1%-2%，占所有神经内分泌肿瘤的20%-30%。2004年世界卫生组织的现行分类将它们分为典型类癌（TC）、非典型类癌（AC）、大细胞神经内分泌癌（LCNEC）和小细胞癌（SCLC）。主要的神经内分泌生物标志物是嗜铬粒蛋白A、突触素和CD56。TC的有丝分裂率较低，<2个有丝分裂/2mm²（10个高倍视野），而AC的有丝分裂率为2-10个有丝分裂/2mm²（10个高倍视野）。Ki-67染色有助于将典型和非典型类癌与高度恶性的LCNEC和SCLC区分开来。临床上，患者通常表现为咳嗽、咯血或支气管阻塞。类癌或库欣综合征以及肿瘤相关性肢端肥大症的发生较为罕见。根治性淋巴结清扫的手术切除是实现长期生存的首选治疗方法。对于无法手术的支气管内局限性肿瘤，内镜切除支气管内肿瘤生长是一个很好的选择。肽受体放射性核素治疗（PRRT）是转移性或不可切除的肺神经内分泌肿瘤患者的一种有前景的治疗选择。在许多先前的研究中，正在测试使用血管生成抑制剂、mTOR抑制剂和酪氨酸激酶抑制剂的新型靶向治疗的有效性。典型类癌肿瘤的转移频率低于AC，TC患者的5年生存率超过90%。AC患者的5年生存率在35%至87%之间。另一方面，高度恶性的LCNEC和SCLC的5年生存率分别在15%至57%之间和<5%。治疗选择和诊断程序的不断增加需要多学科方法以及在多学科肿瘤会议中进行决策，以确保个性化的治疗方法。因此，肺神经内分泌肿瘤患者应在专科中心接受治疗。
