胰腺导管内嗜酸性乳头状瘤的临床病理学特征(附5例报告)

Clinicopathological features of pancreatic intraductal oncocytic papillary neoplasm: a report of 5 cases

目的:探讨胰腺导管内嗜酸性乳头状瘤(pancreatic intraductal oncocytic papillary neoplasm,IOPN)的临床病理学特征。方法:我院5例IOPN,女3例,男2例,年龄(60.0±10.4)岁。分析临床特征、肿瘤大体和镜下表现特点、免疫表型及预后。结果:2例伴腹部隐痛,其中1例半年内体重减轻5 kg。3例体检发现。胰腺肿瘤位于头部2例,位于头体部、颈部、体部各1例。1例血清CA724升高,其他血清学指标均正常。肿瘤呈多房囊性、囊实性或实性。实性区灰白色、质脆,囊内壁光滑或伴乳头样突起。镜下见导管内生长,囊内具有复杂的分支乳头状结构。乳头衬覆的肿瘤细胞胞质丰富,富含嗜酸性颗粒。细胞异型性大,核仁明显。肿瘤细胞间可见杯状细胞,黏液分泌不显著。免疫组织化学结果为,细胞角蛋白7阳性(3/4),MUC1、MUC6阳性(4/4),MUC5AC阳性(3/3),肝细胞抗原Hep Par 1阳性(3/4)。个别肿瘤细胞CK20或MUC2阳性,SYN及CgA阴性(2/2)。其中4例获得随访,均无复发及转移。结论:IOPN是较罕见的胰腺低度恶性上皮性肿瘤。IOPN从胰腺导管内乳头状黏液瘤中分离出来,具有独特的临床及病理学特点。由于IOPN罕见,需进一步研究和加强认识。

Objective To analyze the clinicopathological features of pancreatic intraductal oncocytic papillary neoplasm(IOPN).Methods Five cases with pancreatic IOPN were in Ruijin Hospital including 3 female and 2 male,and the average age(60.0±10.4)years old.The clinical features combined with gross and microscopic pathology,immunophenotype and prognosis were analyzed.Results Two cases had abdominal pain including 1 case losing 5 kg during the period of 6 months.Three patients were found on physical examination.Pancreatic tumor in 2 cases located at the head,in 3 cases at the head and the body,the neck,and the body,respectively.The test showed serological CA724 elevated in 1 case,and other serological indexes were normal.The gross pathology showed that the tumor was multilocular cystic,cystic solid or solid.The solid area was gray-white,fragile,and the inner wall of the cyst was smooth or with papillary protrusions.The microscopic pathology showed that the tumor was ductal growth with complex branched papillary structure in the capsule.The tumor cells lining the papilla had eosinophilic granules,and the cell was atypia and contained typical nucleolus.Goblet cells could be seen among tumor cells.Mucus secretion was present and without significance.Immunohistochemistry showed cytokeratin 7 positive(3/4),MUC1 and MUC6 positive(4/4),MUC5AC positive(3/3),hepatocyte antigen(Hep Par 1)positive(3/4).CK20 or MUC2 in individual tumor cells were positive and SYN and CgA negative(2/2).There were 4 cased with following-up without recurrence or metastasis in any case.Conclusions IOPN is a rare pancreatic epithelial tumor with low malignancy,which is divided from pancreatic intraductal papillary mucinous neoplasms,and has unique clinical and pathological characteristics.Further study with recognition is needed for rare disease of IOPN.