原发性浅表食管小细胞神经内分泌癌15例的临床病理学特征

Primary superficial small cell neuroendocrine carcinoma of esophagus： cUnicopathologic and immunohistochemicai analysis of 15 cases

目的分析原发性浅表食管小细胞神经内分泌癌的临床病理学及免疫组织化学特征，以寻找浅表食管小细胞神经内分泌癌合适的诊断标志物及治疗方法。方法回顾性分析15例原发性浅表食管小细胞神经内分泌癌的临床病理学特征，并采用免疫组织化学MaxVision方法检测嗜铬粒素A（CgA）、神经元特异性烯醇化酶（NSE）、突触素、CD56、甲状腺转录网子－1（TTF—1）、细胞角蛋F1AE1／AE3、CK34S3E12及CK10／13在食管小细胞神经内分泌癌中的表达。结果原发性浅表食管小细胞神经内分泌癌的发病率占同期浅表食管癌的4．8％（15／312）。患者中位生存时间19个月，平均生存时间23．7个月。1、2和5年生存的分别有10、5和1例。免疫组织化学标志物NSE、突触素、AE1／AE3均为阳性，CD56阳性14例，TTF－1阳性9例，CgA阳性8例，CK3413E12阳性1例，CK10／13均为阴性。结论原发性浅表食管小细胞神经内分泌癌恶性度高，进展快速，预后很差。建立在放化疗联合外科切除基础上的系统性治疗或许有助于延长患者的生存期。NSE、突触素、AEI／AE3、CD56、TTF－1、CgA任食管小细胞神经内分泌癌中的高表达对其诊断及鉴别诊断具有一定应用价值。

Objective To study the e.linicopathologie and immunohistochemieal features of primary superficial esophageal small cell neuroendocrine carcinoma （ESCNC）. Methods The clinical, pathologic and immunohistochemical features were retrospectively analyzed in 15 cases of superficial ESCNC. An immunohistochemical study for chromogranin A, neuron-specific enolase, synaptophysin, CD56, TTF-1, 3413E12, AEI/AE3, and CK10/13 was performed. Results Superficial ESCNC accounted for 4, 8% （ 15/ 312） of all cases of superficial carcinoma of the esophagus encountered during the same period. The median survival time was 19 months and the mean survival time was 23.7 months after diagnosis. The one, two and five-year survival rates were 10/15, 5/15 and 1/15, respectively. The immunophenotypie profile was as follows： neuron-specifie enolase （15/15）, synaptopbysiu （15/15）, AE1/AE3 （15/15）, CD56 （14/15）, TTF-1 （9/15）, ehromogranin A （ 8/15 ）, 34[3E12 （ 1/15 ） and CK10/13 （0/15） . Conclusions Superficial ESCNC is a rare and aggressive malignant tumor with poor prognosis. Surgical resection coupled with post-operative ehemoradiotherapy is the mainstay of treatment. The immunohistoehemieal study is valuable in pathologic diagnosis and differential diagnosis.