阑尾管状类癌2例报道及文献复习

Tubular carcinoid of appendix:a report of two cases and review of literature

目的探讨阑尾管状类癌的临床病理特点及免疫表型。方法对2例阑尾管状类癌进行组织学观察及免疫组化分析,并复习文献。结果2例阑尾管状类癌均为女性,分别为16岁和23岁,临床表现均为右下腹痛,术前均诊断为急性阑尾炎,肿瘤均位于阑尾尖部,直径分别为2mm和3mm,组织学上肿瘤细胞异型性小,排列成不连续的管状和短索状结构。免疫表型:CD56、NSE及CgA均呈局灶性阳性,Syn及CK20均为阴性,1例CK7为局灶性阳性,CEA均为弥漫性阳性。随访均为无瘤生存。结论阑尾管状类癌极为罕见,体积很小,上皮及神经内分泌免疫标记均有表达,局部切除即可治愈,须与转移性腺癌鉴别诊断。

Purpose To investigate the clinico-pathological features and immunological phenotypes of tubular carcinoid of appendix. Methods Two cases of tubular carcinoid of appendix were analyzed by histology and immunohistochemistry with review of the literature. Results Two patients were female aged 16 and 23 years old. Both patients presented right lower abdominal pain and were diagnosed as acute appendicitis preoperatively. The tumors were located at the tip of appendix and were measured 2mm and 3mm in diameter respectively. Tumor cells with slight atypia arranged in discrete tubules and short cords in histology. CD56, NSE and CgA were focally positive in both cases. Syn and CK20 were negative. CK7 was focally positive in one case. CEA was diffusely positive. Follow-up was performed. Both patients were alive free of this disease. Conclusions Tubular carcinoid of appendix is very rare neoplasm with small size positive for epithelial and neuroendocrine markers. Local excision is curative. It should be differentiated from metastatic adenocarcinoma.