胃肠胰神经内分泌瘤的诊断与治疗

The diagnosis and therapeutics of gastroenteropancreatic neuroendocrine tumors

神经内分泌瘤（NET）是起源于神经内分泌细胞的恶性实体肿瘤,发病率在过去30年内增加了5倍。消化道是NET最常见原发部位,胃肠胰神经内分泌瘤（GEP-NET）发病率在消化道肿瘤中排名第二,是胰腺癌的2倍。GEP-NET是一个进展性疾病,发生转移者中位生存时间仅33个月。GEP-NET可分为功能性和无功能性两类,早期症状不典型,从起病到明确诊断的时间为5~7年。早期诊断对GEP-NET的治疗至关重要。嗜铬粒蛋白A（Cg A）是目前诊断NET最敏感和最特异的标志物。生长抑素受体成像也是重要诊断方法。GEP-NET目前尚无统一的治疗规范,手术切除是唯一能达到根治的手段。化疗是晚期GEP-NET一线选择,常用药物有卡培他滨、替莫唑胺等。生长抑素类似物如长效奥曲肽对控制症状效果显著。近年来靶向药物舒尼替尼和雷帕霉素在GEP-NET的临床研究中显示出很好的疗效。

Neuroendocrine Tumor （NET） is a kind of malignant solid tumors, who is originated in the neuroendocrine cells. The incidence has increased 500% over the last 30 years, and the most common diseased parts is digestive tract. Gastro-Entero-Pancreatic NET is the second most prevalent GI malignancy and two times more than pancreatic cancer. GEP-NET is a progressive disease, the median survival for patients with advanced GEP-NET is 33 months. GEP-NET can be functional or non-functional and has no specific symptoms in the early period. The time from onset to diagnosis is 5 to 7 years. Early diagnosis is important for the treatment of GEP-NET, CgA is the most sensitive and specific tumor marker to diagnose GEP-NET. Somatostatin receptor scintigraphy is also important diagnostic method. There is no consensus guidelines for the treatment of GEP-NET, surgical resection is the only best treatment. Chemotherapy is a treatment option for patients with advanced GEP-NET, such as Capecitabine, Temozolom-ide. Somatostatin analogues such as Octreotide have significant effect of controlling symptoms, and targeted drugs such as Sunitinib, Everolimus also have very good curative effect in clinical research.