胰腺实性假乳头状瘤的研究进展.docVIP

胰腺实性假乳头状瘤的研究进展 　　摘 要 胰腺实性假乳头状瘤（solid pseudopapillary tumor of pancreas，SPTP）是少见的胰腺低度恶性肿瘤，以年轻女性多见，病因及发病机制尚不清楚。SPTP临床表现不典型，常表现为腹痛或腹部肿块伴或不伴消化系统症状。SPTP的影像学表现为囊性或囊实性占位，境界清楚，包膜完整，无胰管和胆管扩张，且无内分泌功能异常。病理学发现SPTP由实性区、囊性区和假乳头状结构组成。SPTP术前定性诊断有一定困难，容易误诊，外科手术是首选的治疗方法，且多预后良好，转移复发率低，放疗和化疗敏感性尚不清楚。本文综述近年来SPTP的研究现状。 　　关键词 实性假乳头状瘤 胰腺 诊断 治疗 　　中图分类号：R657.5+2 文献标识码：A 文章编号：1006-1533（2016）08-0008-05 　　Advance in the research of solid pseudopapillary tumor of pancreas 　　LI Qiao， HUA Rong， SUN Yongwei 　　（Department of Pancreatobiliary Sugery， Renji Hospital affiliated to Shanghai Jiao Tong University， Shanghai 200001， China） 　　ABSTRACT Solid pseudopapillary tumor of pancreas （SPTP） is a rare low-grade malignant tumor and primarily affects the young females. The etiology and pathogenesis of SPTP are still unclear. The SPTP， whose clinical manifestation is not typical， is characterized by abdominal pain and mass with or without symptoms of the digestive system. Imaging examinations show that the SPTP is cystic or solid capsule placeholder with clear state and complete envelope， free of the pancreatic duct and bile duct expansion as well as endocrine dysfunction. Pathologic findings demonstrate that SPTP is consisted of solid tumor， cystic papillary and false architecture. Since the qualitative diagnosis of SPTP is difficult before operation， it is easily misdiagnosed. Surgery is the preferred treatment with well prognosis and low recurrence. The sensitivity of SPTP to radiation and chemotherapy treatment remains unknown. This paper reviews the study of STPT in the recent years. 　　KEY WORDS solid pseudopapillary tumor of pancreas； pancreas； diagnosis； treatment 　　胰腺实性假乳头状瘤（solid pseudopapillary tumor of pancreas，SPTP）是少见的胰腺低度恶性或潜在恶性肿瘤，以青年女性多见，表现为胰腺肿瘤中央组织坏死而囊性变，周围残存肿瘤组织出现假乳头状结构。本文综述近年来SPTP的研究现状。 　　1 病因及发病机制 　　SPTP的病因和发病机制尚不清楚，有文献报道其与b-catenin基因突变有关。b-catenin是一种多功能蛋白，与上皮细胞钙黏蛋白结合，在细胞-细胞黏附中起重要作用，在Wnt信号转导通路的下游基因转录中起催化作用，其异常表达可导致Wnt信号转导通路异常激活，相关基因表达改变，是SPTP发生的重要环节[1]。E-Bahrawy等[2]发现b-cateni