Russo S, Russo F, Maiello F M, Paolini B, Carrabba A, De Gregorio A
Pathology Dpt., Maresca Hospital, Torre del Greco, Napoli, Italy.
Pathologica. 2012 Aug;104(4):185-9.
We report a case of primary combined large cell neuroendocrine carcinoma (LCNEC)--pure mucinous carcinoma of the gallbladder (MANEC)--which represents the first description of this entity.
The patient is a 59-year-old Italian male who underwent cholecystectomy under a preoperative diagnosis of cholecystitis with gallstones and gallbladder tumour. During laparotomy, cholecystectomy, liver wedge resection and regional lymph node dissection were performed. The resected gallbladder showed a thickened wall, gallstones and a 4 cm gelatinous, cauliflower-like soft tissue mass.
Following surgery, the gallbladder tumour was diagnosed as a mixed endocrine-exocrine carcinoma. There was evidence of lymph node metastasis or direct liver invasion. The mucin-producing carcinoma was composed of poorly differentiated glandular cells with mucin lakes. The LCNEC was characterized by large cells with prominent nucleoli, coarse chromatin and a high mitotic rate. The cells showed an "organoid" growth pattern with rosette formation and frequent areas of necrosis. Chromogranin A, synaptophysin and CD56 were diffusely and strongly expressed.
This case may provide helpful insights regarding the histogenesis of this unusual combination of tumors: the concept of a collision tumor between two neoplasms that have arisen in adjacent areas may be the best explanation for its pathogenesis.
我们报告了一例原发性混合性大细胞神经内分泌癌（LCNEC）——胆囊纯黏液腺癌（MANEC），这是对此实体的首次描述。
患者为一名59岁的意大利男性，因术前诊断为胆囊炎伴胆结石及胆囊肿瘤而接受胆囊切除术。剖腹手术期间，进行了胆囊切除术、肝楔形切除术和区域淋巴结清扫术。切除的胆囊显示壁增厚、有胆结石以及一个4厘米的胶冻状、菜花状软组织肿块。
手术后，胆囊肿瘤被诊断为混合性内分泌-外分泌癌。有淋巴结转移或肝脏直接侵犯的证据。产生黏液的癌由具有黏液湖的低分化腺细胞组成。LCNEC的特征是细胞大，核仁突出，染色质粗糙，有丝分裂率高。细胞呈“器官样”生长模式，有玫瑰花结形成且常有坏死区域。嗜铬粒蛋白A、突触素和CD56弥漫性强表达。
该病例可能为这种不寻常的肿瘤组合的组织发生提供有用的见解：两个在相邻区域发生的肿瘤之间碰撞瘤的概念可能是其发病机制的最佳解释。
