原发性食管神经内分泌癌影像学及病理特征

目的：探讨原发性食管神经内分泌肿瘤的病理特征及临床诊断模式。方法：回顾性分析2001年1月至2010年12月在本科接受治疗的32例原发性食管神经内分泌癌的临床影像学诊断方法、病理特征。结果：32例原发性食管神经内分泌癌中，小细胞癌27例（84．38％，27／32），不典型类癌4例（12．5％，4／32），类癌1例（3．13％，1／32）；影像学（螺旋CT或PET／CT检查）评估可手术患者的临床TNM分期（TNMstaging），手术后病理TNM分期（Pathological TNM staging，pTNM分期）上调的占本组患者总数的12．5％（4／32），主要为3例术前评估为L的患者术中见为侵犯外膜（T4），另1例术前为T，NOM0患者术后有单站纵隔淋巴结转移（N1）；免疫组化提示肿瘤细胞表达嗜铬素A（CgA）、肿瘤细胞表达突触素（Syn）、神经特异性烯醇化酶（NSE）等单种或多种分化标记物呈阳性表达。结论：原发性食管神经内分泌癌特别是小细胞癌恶性程度高，综合多部位的增强CT或PET／CT等多种影像学检查手段有助于临床分期，病理检查中联合CgA、Syn、NSE等多种分子标记有助于明确诊断。

Analysis of imaging and pathological features of primary esophageal neuroendocrine carcinoma

Objective：To explore the pathological features and clinical diagnostic modalities of primary esophageal neuroendocrine carcinoma through analysis of 32 cases with primary esophageal neuroendocrine carcinoma. Methods：Between January 2001 and December 2010, a retrospective analysis was performed on 32 patients with primary esophageal neuroendocrine carcinoma registered in our department so as to summarize the clinical imaging diagnostic methods and pathological features. Results： Of 32 patients with primary esophageal neuroendocrine carcinoma, small cell neuroendoerine carcinoma was found in 27 cases （ 84. 38% , 27/32 ） , atypical earcinoid in 4 cases （12.5% ,4/32） and carcinoid in 1 case （3. 125%, 1/32）. Imaging studies （spiral CT or PET/CT） were used to evaluate the TNM staging in operable patients. Up-regulation of TNM staging as revealed by postoperative pathology was found in 12.5% （n =4/32） of all patients, mainly involving 3 cases upregulated from T3 before surgery to T4 with outer membrane invasion at surgery and 1 NOMO case up-regulated from T2 to N1 with single-station mediastinal lymph node metastasis. Immunohistochemistry indicated that single or multiple differentiation markers such as chromogranin A （CgA）, synaptophysin （Syn） and neuron-specific enolase （NSE） were positively expressed by tumor cells. Conclusion： Primary esophageal neuroendocrine carcinoma has a high degree of malignancy, especially small cell carcinoma. The various imaging examinations such as multi-site enhanced CT or PET / CT may be helpful for the clinical staging. In addition,the pathological examination combined with multiple molecular markers, such as CgA, Syn and NSE, contributes to definite diagnosis.