胰腺神经内分泌肿瘤课件.pptVIP

胰腺神经内分泌肿瘤 中山大学附属第三医院肿瘤内科 陈展洪 2011年10月19日 胰腺神经内分泌肿瘤的治疗进展 局限期肿瘤手术原则 尽可能切除肿瘤病灶 伴有多发性内分泌瘤病1型、卓艾综合症的肿瘤应行whipple手术治疗 其余情况多采用局部切除或摘除 暂无辅助治疗临床试验数据 Sunitinib RAD001：依维莫斯 PFSOS Toxicity：Aware of pneumonitis Acceptable safety profile: stomatitis, rash, infection, infrequent pneumonitis 结论 依维莫斯显著延长PFS（延长6.4m） 可用做进展的低或中分级的胰腺神经内分泌肿瘤的标准治疗 注意肺毒性 Peptide receptor therapy:PPRT多肽受体放射性核素治疗 肝转移灶的处理 最常见的死亡原因：肝转移 治疗方案：肝叶切除、不规则的转移瘤切除术、术中射频消融或冷冻治疗、多种方法联合 介入栓塞 Take home message 胰腺神经内分泌肿瘤，散发，约占胰腺恶性肿瘤3-5%，发病率呈上升趋势。 多伴有激素异常释放。经常转移到肝脏。 预后较胰腺癌好，局限期、局部晚期、晚期mOS：124m,70m, and 23 m 尽可能切除肿瘤病灶；伴有多发性内分泌瘤病1型、卓艾综合症的肿瘤应行whipple手术治疗；其余情况多采用局部切除或摘除。 积极处理患者肝转移病灶可能改善生存 晚期患者多采用生长抑素、化疗或靶向治疗 生长抑素ORR低4%，以缓解症状、稳定肿瘤为主 化疗有效的药物：链脲霉素+5Fu+ADM、替莫唑胺（或DTIC）联合卡培他滨、CIS/L-OHP+VP-16 靶向治疗：舒尼替尼、依维莫斯，延长PFS为主 注意依维莫斯肺毒性 Stage and survival of 1157 patients from the time of diagnosis. Age, location and stage correlated with survival. Adjusted for stage, pancreatic tail lesions do better than head. May be due to location of different tumors, ie VIPomas and insulinomas more likely in tail? Median duration of survival of patients with localized (n = 167), regional (n = 289), and distant disease (n = 558) was 124, 70, and 23 months, respectively (P .001). Of the 1310 cases, 125 (10%) were not staged (Table 2). For the remaining 1185 cases, 179 (14%) were localized; 295 (23%) were classified as regional; and 711 (54%) were classified as distant. * 卓艾综合症又称佐林格一埃利森综合征(Zollinger-Ellison syndrome),来源于G细胞， 是一种以消化道溃疡为主要表现的综合病症.在胰腺内分泌瘤中发病率仅次于胰岛素瘤。60%~70%为恶性，常伴有淋巴结或肝转移。25%-30%的病人同时存在其他内分泌肿瘤〔多发性内分泌瘤病I型(MEN I)]。部分肿瘤位于胰腺外，十二指肠为其好发部位。病因为胰岛D细胞的胃泌素瘤.由于肿瘤能分泌大量胃泌素,刺激胃酸分泌极度增加,导致顽故难治的不典型溃疡,容易出血和穿孔.常伴有甲状旁腺或垂体腺瘤及其相应症状. * Somatostatin analog High affinity for sst2 and sst5 Moderate affinity for sst3 Can give up to 500 ug tid. Binds to receptor and gets internalized. Also mention similar somatostatin analog, lanreotide. The sst are coupled to cAMP and decreased calcium influx and generally inhibit hormonal secretion and intestinal motility