胰腺内分泌肿瘤的研究进展

Recent advances in understanding pancreatic endocrine tumors

胰腺内分泌肿瘤(pancreati cendocrine tumors,PETs)是一类少见肿瘤,发病率约占总人口的4-5/100万,占胰腺肿瘤的1%-2%,病程缓慢,最终发生转移致死.根据其临床表现,PETs分为功能性和无功能性两类.CD10、CD44、CD99、p27、COX2、Ki-67、KIT、CK19、ARHI、RUNX1T1、survivin等基因表达异常、染色体杂合缺失、抑癌基因甲基化及Ghrelin过度表达等因素在PETs的发病机制中发挥重要作用.铬粒素A(CgA)已作为PETs重要的血清学标志物.KIT和内皮蛋白是PETs新的独立预后指标.CgA的组织病理学阳性结果以及胃泌素、胰岛素原、血管活性肠肽(VIP)、胰高糖素等特异性激素检测可作为PETs的病理和定性诊断依据.此外,除标准的定位程序外,计算机断层扫描(CT)、正电子发射断层成像与计算机断层扫描(PET/CT)、磁共振成像(MRI)、超声(US)、超声内镜(EUS)、腹腔镜超声(LUS)、动态增强螺旋CT扫描、选择性动脉内刺激试验(ASVS)及生长抑素受体闪烁成像(SRS)等技术也用于PETs的定位诊断.外科手术仍是PETs治疗的首选.腹腔镜及生长抑素类似物也已常规应用.上述最新进展为PETs的早期诊断和治疗提供了新的思路.

Pancreatic endocrine tumors（PETs） are uncommon and have an incidence of approximately 4-5 per 1 000 000 people,accounting for 1%-2% of all pancreatic neoplasms.They usually grow slowly,eventually metastasize and lead to death.PETs can be classified as functioning or non-functioning tumors based on clinical manifestation.The pathogenesis of PETs may involve abnormal expression of CD10,CD44,CD99,p27,COX2,Ki-67,KIT,CK19,ARHI,RUNX1T1,and survivin genes,loss of heterozygosity on chromosomes,hypermethylation of tumor suppressor genes,and overexpression of ghrelin.Chromogranin A（CgA） has long been used as an important broad-spectrum marker for the identif ication of PETs.KIT and endoglin are new independent prognostic markers for PETs.The diagnosis is based on histopathology demonstrating neuroendocrine features such as positive staining for chromogranin A and specifichormones such as gastrin,proinsulin,vasoactive intestinal peptide（VIP） and glucagon.In addition to standard localization procedures,radiology diagnosis including computed tomography（CT）,positron emission tomography and computed tomography（PET/CT）,magnetic resonance imaging（MRI）,ultrasound（US）,endoscopic ultrasound（EUS）,laparoscopic ultrasound（LUS）,dynamic enhanced spiral CT,selective arterial stimulation and venous sampling（ASVS）,and somatostatin receptor scintigraphy（SRS） are performed.Surgery is still one of the cornerstones in the management of PETs.Laparoscopy,and drugs of somatostatin analogs are routinely used.Understanding of the recent advances of PETs has important implications for the early diagnosis and treatment of PETs.