腹膜浆液性乳头状腺癌4例并临床病理分析

Primary peritoneal papillary serous carcinoma: a clinicopathological observation of 4 cases and literature review

目的探讨腹膜浆液性乳头状腺癌(peritoneal papillary serous carcinoma,PPSC)的临床病理学特征、免疫表型、鉴别诊断及预后。方法对4例PPSC行免疫组化SP法染色及HPV检测,并复习相关文献。结果 4例PPSC均为女性,年龄23~62岁,平均51岁。临床表现多为无特异性的腹部不适,CA125升高,影像学表现为腹腔肿块。镜下肿瘤细胞形成形态不一、大小不等的乳头状结构;肿瘤细胞为低柱状,核质比大,核圆形,核仁大,异型性明显,核分裂象易见。腺体及间质内可见砂砾体。免疫表型:肿瘤细胞CA125、WT1、CK7、p53、CEA、p16均呈阳性,部分肿瘤细胞表达CK5/6、ER、PR;不表达GCDFP-15、CR、D2-40、CK20、Villin、CDX2、TTF1;HPV检测阴性。结论 PPSC是一种少见的原发于腹膜的恶性肿瘤,临床误诊率高,依据其临床和病理组织学特点,结合免疫组化染色可以明确诊断。

Purpose To study the clinicopathological features, immunophenotypes and differential diagnosis of peritoneal papillary serous carcinoma （PPSC）. Methods Four cases of PPSC were studied by clinical and pathological data, immunohistochemical SP staining, HPV detection and literatures were reviewed. Results All patients were female （mean age 51 years, range from 23 to 62 years）. The clinical manifestations were nonspecific abdominal discomfort, increased CA125, abdominal mass. The histology shows a different size of the papillary structure. The tumor cells were low columnar with high karyoplasmic ratio, round nucleus, large nucleoli, atypia and mitoses are easy to see. There is gravel body in the stroma and gland. Immunohistoehemistry, CA125, WT1, CK7, p53, CEA and p16 were positive expressed in tumor cells, and some tumor cells expressed CK5/6, ER, PR. GCDFP-15, CR, D2-40, CK20, Villin, CDX2, TI^F1 expression were negative. HPV was detected as negative. Conclusion PPSC is a rare malignant tumor with a high misdiagnosis rate. It can be diagnosed clearly by immunohistochemical staining based on clinical and pathological features.