摘要目的 分析肺支气管扩张症伴发肺神经内分泌细胞增生及微小瘤形成病例的临床病理特征.方法 收集22例此类病例,通过光镜下观察及免疫组织化学EnVision法检测分析其临床病理特征及免疫组织化学特点.结果 22例肺支气管扩张症伴发神经内分泌细胞增生及微小瘤形成患者平均年龄53岁,男女比例9∶13,大体检查为典型的支气管扩张症表现,仅1例切面可见灰白色质软结节,直径＜5 mm;镜下观察22例均有支气管扩张症表现,在此基础上均可见细小气道上皮细胞下神经内分泌细胞增生并形成微小瘤(直径均＜5 mm),10例伴单发微小瘤形成,12例伴多发微小瘤形成,平均直径1.6 mm,淋巴结均未发现特殊改变.术后16例分别随访17～117个月,平均随访58个月,均无类癌发生,1例合并肺癌患者术后猝死,5例失访.免疫组织化学嗜铬粒素A( CgA,18/18)、突触素(16/16)、AE1/AE3(16/16)、TTF-1(14/15)、CD56( 14/14)均阳性,12例Ki-67阳性指数均＜2％.结论 免疫组织化学CgA、突触素、CD56、TTF-1、AE1/AE3有助于诊断.早期发现、手术切除和密切随访有助于阻止疾病进展.

abstractsObjective To study the clinical and pathological features of pulmonary neuroendocrine cell hyperplasia and tumodets with bronchiectasis. Methods Both the clinicopathologic changes and immunohistochemical findings were examined with microscopy and EnVision method in 22 cases of pulmonary neuroendocrine cell hyperplasia and tumorlets.Results The average age of the 22 patients was 53 years,with a male to female ratio of 9∶13.On macroscopic examination the lungs showed bronchiectasis; one case was accompanied by gray-white,soft nodules (diameter ＜ 5 mm).Microscopy of the HE sections showed the basic pathologic change was bronchiectasis,accompanied by neuroendocrine cell hyperplasia and tumorlet formation in the pulmonary parenchyma surrounding the bronchioles,presenting as single nodule (10 patients),or multifocal nodules (12 patients),with average size of 1.6 mm in diameter.No tumor cells were identified in the lymph nodes.Sixteen of 22 patients were disease-free after an average follow-up period of 58 months (17-117 months); one patient died suddenly after surgery; and five were loss of follow up.Immunohistologically,the tumor cells were positive for CgA (18/18),Syn (16/16),AE1/AE3 (16/16),TTF-1 (14/15), and CD56 (14/14), and Ki-67 index was ＜ 2％ in 12 cases. Conclusions Immunohistological staining for CgA,Syn,CD56,TTF-1 and AE1/AE3 can confirm the diagnosis.Early detection,pulmonary resection and follow-up help prevent the progression of these diseases.