阑尾杯状细胞类癌7例临床病理分析

Appendiceal goblet cell carcinoid: a clinicopathological study of 7 cases

目的探讨杯状细胞类癌（GCC）的临床病理特点。方法复习7例手术切除GCC病例,分析其临床资料、组织学、免疫表型及随访情况,结合相关文献进行讨论。结果 7例GCC中,女性2例,男性5例,平均年龄54岁,均为弥漫腹腔种植Ⅳ期,但首发症状不典型,3例为常规体检发现腹腔积液就诊。7例局灶均见经典GCC形态：印戒样细胞成巢团样排列浸润阑尾浆膜层,其中3例局灶排列呈不规则片状,核异型性显著。1例无伴发病变,独立引起腹膜假黏液瘤,黏液分布于细胞周围;余6例均伴发其他病变,其中1例伴发高分化神经内分泌肿瘤G1及血吸虫,另5例均伴有黏液腺癌。免疫组化显示神经内分泌标记均（＋）,Ki-67阳性率4%～71%,2例p53部分（＋）。随访4～18个月,2例因腹膜炎及肠梗阻死亡,5例带瘤生存。结论 GCC是一种在组织形态和免疫表型上具有外分泌和内分泌双重特点的肿瘤,临床症状不典型,应及时行病理确诊。GCC易伴发其他病变,黏液湖的分布方式能为其鉴别诊断提供一定依据,其与印戒细胞癌不易区分。

Objective To investigate the clinico-pathological characteristics of the goblet cell carcinoid（ GCC）.Methods The clinical manifestations,histology,immunohistochemistry and follow-up were retrospectively analysed in 7cases of GCC and related literatures were reviewed. Results In 7 cases of GCC,2 females and 5 were males,the average age was 54 years old. Although all patients with diffuse abdominal cavity implantation,WHO stage Ⅳ,the first sympotom was not typical. Even there are 3 patients diagnozed with ascites by routine physical examination. GCC was composed of uniform goblet cells which formed a nest pattern and infiltration into the appendiceal serosa,of which we found3 cases arranged in irregular flakes,marked nuclear atypia. All cases were accompanied with other diseases except 1 case which also caused peritoneal pseudomyxoma independently. 1 case was associated with neuroendocrine tumor G1,the other5 cases were associated with mucinous adenocarcinoma. Immunohistochemistry showed that the neuroendocrine markers were positive,the positive rate of Ki-67 were 4%- 71%,and only 2 canses showed P53 were focal positive. Patients were followed up for 4- 18 months; 2 cases died of peritonitis and intestinal obstruction,5 cases survived with tumor.Conclusion GCC has double characteristics of exocrine and endocrine tumors in mrophology and immunotype. The clinical symptoms of GCC are not typical. Pathological examination is necessary. GCC is commonly associated with other diseases. The distribution of mucous can provide basis for the differential diagnosis. It is difficult to distinguich from signet ring cell carcinoma.