胰母细胞瘤临床病理特征分析

Analysis of Clinicopathological Features of Pancreatoblastoma

目的探讨胰母细胞瘤的临床病理学特点及诊断要点。方法回顾性分析1例胰母细胞瘤肝多发转移的临床病理资料,并结合文献对其诊断及鉴别诊断进行分析。结果胰母细胞瘤肝转移。镜检:肝组织内见索条状、地图状排列的肿瘤组织,肿瘤细胞排列成边界清楚的不规则岛状,由纤维间质分隔,低倍镜下呈"地图样"外观,部分呈腺泡状,局部形成腺腔,可见鳞状小体。免疫组织化学染色结果:CK7(-),CK20(-),Villin(-),CK8(+),CK18(+),CK19(+),EMA(+),Cg A(-),Syn(-),Ki-67(阳性率约5%),AFP(-),α-ATT(+),Hepatocyte(肿瘤细胞-,肝细胞+)。病理诊断为胰母细胞瘤。术后未进行放化疗,随访14个月患者再无复发。结论胰母细胞瘤是1种好发于儿童,偶发于成人,罕见的胰腺恶性外分泌肿瘤,鳞状小体为其组织学特征性改变,具有多向分化的特征,其可能起源于原始多潜能细胞,应与其他胰腺肿瘤鉴别,其预后比成人胰腺癌好。

Objective To investigate the clinicopathological features of pancreatoblastoma and diagnosis.Methods Clinical data of 1 cases of multiple metastatic pancreatoblastoma was retrospectively studied,and treatment and differential diagnosis were analyzed.Results Microscopic examination of pancreatoblastoma liver metastasis：liver tissue in strip map,arranged in the shape of tumor tissue,tumor cells arranged in a clear boundary of irregular island,separated by fibrous stroma,low magnification was ＂ map＂ appearance,part in acinar,local formed glandular cavity,visible squamous corpuscles.The results of immunohistochemical staining：CK7（-）,CK20（-）,Villin（-）,CK8（＋）,CK18（＋）,CK19（＋）,EMA（＋）,Cg A（-）,Syn（-）,Ki-67（positive rate 5%）,AFP（-）,α-ATT（＋）,Hepatocyte（tumor cells,liver cells ＋）.Pathological diagnosis was pancreatoblastoma.Patients did not receive postoperative radiation and chemotherapy,followed up for 1 years without recurrence.Conclusion Pancreatoblastoma is mainly see on children,and seldom on adults,it is a rare malignant exocrine pancreatic cancer,squamous corpuscles are its histological characteristic change,it has the feature of multi-directional differentiation,which may originate from primitive pluripotent cells,it should be distinguished from other pancreatic tumor during diagnosis,its prognosis is better than adult pancreatic cancer.