转移性胰腺神经内分泌肿瘤综合治疗

胰腺神经内分泌肿瘤是一类少见的肿瘤,除胰岛素瘤外,大部分在诊断时就合并远处转移。对无手术禁忌证的病人,建议手术同时切除原发灶和转移灶;对不适合手术的病人,可选择介入栓塞/化疗、射频消融,配合化疗和生长抑素类药物的治疗。综合治疗可使转移病人的5年存活率达到60%~80%,2年症状控制率达到90%。胰腺神经内分泌肿瘤肝转移行肝移植治疗还有待进一步的临床研究验证。     

胰腺神经内分泌肿瘤9例临床分析

目的：探讨胰腺神经内分泌肿瘤（PNEN）临床及病理特点、诊断与治疗方法,为进一步提高该病的认识提供帮助。 方法：回顾性分析2011年12月—2013年12月期间安徽医科大学第一附属医院收治的9例PNEN患者的临床及病理资料。 结果：9例患者中,男3例,女6例;9例均行B超及CT检查,2例行MRI检查。影像学提示肿瘤位于胰头部3例,颈体部1例,胰尾部1例,体尾部3例,体尾部及肝脏膈面发现结节1例。9例患者有不同的临床症状,胰岛细胞瘤1例,可见明显的低血糖症状,具体表现为头晕、乏力,并伴随有嗜睡、饥饿感、神志不清,其余患者无典型症状;所有患者均接受手术治疗,均经术后病理及免疫组化证实。 结论：PNEN发病率低,起病隐匿,常无典型临床症状和体征,确诊主要依靠病理活检及免疫组化,目前手术治疗为其首选的治疗方法。     

胰腺神经内分泌肿瘤的腹腔镜治疗分析

目的:探讨胰腺神经内分泌肿瘤的腹腔镜治疗方法及效果。方法:2014年6月至2017年6月采用完全腹腔镜手术治疗胰腺神经内分泌肿瘤5例,其中男3例,女2例,患者32~62岁。无功能胰腺神经内分泌肿瘤3例,2例表现为腹部不适,1例无症状;功能性胰腺神经内分泌肿瘤2例,均为胰岛素瘤,表现为反复发作的低血糖症状。病程1周~5年。结果:5例均成功施行完全腹腔镜胰腺神经内分泌肿瘤切除术,术后经病理证实符合胰腺神经内分泌肿瘤。手术时间65~420 min,出血量50(20,700)ml,输血1例。术后平均住院(6.4±2.3)d。术后胰腺生化瘘2例,经保守治疗后痊愈。无术后出血、腹腔感染等严重并发症发生。结论:腹腔镜手术治疗无功能性胰腺神经内分泌肿瘤具有创伤小、康复快的优点,是安全、有效的治疗方法。对于功能性胰腺神经内分泌肿瘤,在术前、术中精准定位的基础上,也可选择腹腔镜手术治疗。     

胰腺神经内分泌肿瘤初治治疗策略

20％ ～ 30％胰腺神经内分泌肿瘤(pNENs)在初诊时可切除,70％ ～ 80％的pNENs是局部晚期或转移性肿瘤.对手术可切除的pNENs,建议积极手术切除原发肿瘤和转移肿瘤.对功能性的局部晚期或转移性pNENs,积极的减瘤术有助于控制症状,改善患者的生命质量;无功能性肿瘤不推荐减瘤术,除非发生出血、梗阻等危及生命的并发症.对Ⅰ型肝转移,应积极联合原发肿瘤切除;Ⅱ型肝转移,可探索全身综合治疗联合肝脏的局部治疗,如RFA、动脉栓塞和栓塞化疗;Ⅲ型肝转移可给予舒尼替尼、依维莫斯联合长效生长抑素的治疗.pNENs获得根治性切除后无需辅助治疗,但若是转移性病灶获得根治性切除,建议给予后续治疗,预防复发.     

腹腔镜手术治疗胰腺神经内分泌肿瘤的临床疗效

目的 探讨腹腔镜手术治疗胰腺神经内分泌肿瘤(pNENs)的临床疗效.方法 回顾性分析2005年3月至2014年6月浙江大学医学院附属邵逸夫医院收治的31例行腹腔镜手术治疗pNENs患者的临床资料.根据肿瘤位置及大小选择具体术式.采用电话和门诊方式进行随访,随访时间截至2014年8月.结果 31例患者成功完成腹腔镜手术,其中腹腔镜胰腺肿瘤剜除术4例,腹腔镜胰十二指肠切除术2例,腹腔镜中段胰腺切除术2例,腹腔镜保留脾脏胰体尾切除术6例,腹腔镜胰体尾联合脾脏切除术17例.手术时间为(205±74) min,术中平均出血量为125 mL(10～1 000 mL),术中输血率为9.7％ (3/31).术后平均下床活动时间为2d(1 ～3d),术后肛门平均排气时问为3 d(1～4d),术后平均进食流质饮食时间为3d(1 ～7 d),术后平均住院时间为10 d(5 ～63 d).术后并发症发生率为35.5％(11/31),均经对症处理后痊愈.无围手术期死亡患者.31例患者获得术后随访.中位随访时间为21个月(5～111个月).1例患者术后即出现胰腺外分泌功能不足表现;3例患者术后1个月出现糖尿病;1例患者术后1年因肿瘤广泛转移死亡;1例患者术后3年再次出现Whipple三联征,经MRI检查及术中超声证实胰头、胰尾部多发肿瘤.其余患者未出现肿瘤复发或转移.结论 腹腔镜手术治疗pNENs创伤小、恢复快,近、远期疗效好,安全可行.其具体术式选择主要取决于肿瘤的位置和大小.     

胰腺神经内分泌肿瘤8例临床分析

目的:探讨胰腺神经内分泌肿瘤(pNET)的诊疗方法及预后。方法:回顾性分析2008年1月—2017年1月间内蒙古医科大学附属医院收治的8例p NET患者的临床资料。结果:8例pNET患者,男2例,女6例;年龄36~72岁,平均年龄53岁;均行影像学检查明确病灶;5例无功能性肿瘤主要表现为腹痛腹胀、腹部肿块、食欲不振、消瘦,3例功能性肿瘤以低血糖反应或高血糖为主要表现;均行手术治疗,其中1例行肿瘤局部切除术,2例行胰体尾联合切除术,4例行胰十二指肠切除术,1例行胰体尾+脾切除术+胰腺空肠吻合术。8例随访30~66个月,7例存活,1例死亡。结论:结合实验室和影像学检查,有助于该病的诊断;确定合理可行的综合治疗方法,可提高治愈率、改善患者生活质量和预后。     

胰腺神经内分泌肿瘤的手术治疗

胰腺神经内分泌肿瘤(pNENs)分为功能性与无功能性两大类,常见类型包括胰岛素瘤、胃泌素瘤、胰高血糖素瘤、血管活性肠肽瘤等,根治性手术切除是pNENs患者治愈和长期生存的唯一方法.基本手术方式包括局部切除和规则切除两类,前者为肿瘤局部切除(剜除)术,后者包括胰十二指肠切除术(标准或保留幽门)、胰体尾切除术(联合脾脏切除或保留脾脏)及中段胰腺切除术等.肝脏是最常见远处转移部位,手术切除是肝转移瘤首选治疗方法,RFA、TACE以及肝移植可以作为辅助治疗手段.     

胰腺神经内分泌肿瘤诊治进展

胰腺神经内分泌肿瘤(pNET)是一种少见疾病,根据是否伴随相应的内分泌症状分为功能性和无功能性两类。手术是pNET最主要的治疗手段,近年来随着对pNET发病机制的了解,新的诊断技术和治疗手段的出现,以及多学科协作诊治模式的应用,pNET的诊断和治疗发生了巨大的变化,为pNET患者尤其是晚期患者带来了希望。     

胰腺神经内分泌肿瘤外科治疗

胰腺神经内分泌肿瘤种类较多而性质不同,但发生率很低,造成临床处理经验积累缓慢,治疗方式和效果评价存在分歧。无论根治切除肿瘤还是降低肿瘤负荷,外科手术是主要手段。不同起源的疾病类型,不同部位的病灶,不同发展阶段的肿瘤,需要不同术式,也会产生不同疗效。而针对类似病变,由于术式等治疗方式的差异,可影响病人预后。     

胰腺神经内分泌肿瘤的外科诊治

目的探讨胰腺神经内分泌肿瘤的临床特点和外科诊治方法。方法回顾性分析1980年1月至2006年8月经病理确诊的12例胰腺神经内分泌肿瘤的临床资料。结果本组12例患者,男5例,女7例,年龄27～73岁。肿瘤位于胰头部10例,胰颈胰体1例,胰体部1例。临床症状以上腹部疼痛为主。术前血浆CA19-9基本正常,影像学检查易误诊为胰腺癌。12例患者中,行肿瘤切除＋节段性胰腺切除术2例（其中1例加行区域淋巴结清扫术）,行扩大胰头十二指肠切除术3例,经典胰头十二指肠切除术6例,因肝脏广泛转移行肝肿块切除活检＋胆囊切除＋胆总管空肠吻合术1例。术后病理检查：均诊断为胰腺神经内分泌癌,瘤体直径4～14 cm,均呈浸润性生长,淋巴结转移5例,侵犯十二指肠4例,侵犯胆总管3例,侵犯肠系膜上动静脉1例,侵犯下腔静脉1例,肝转移1例。随访9例,8例至今存活,1例术后第8个月死于肝转移。结论对于血清CAl9-9不高且胰腺占位体积较大的胰腺肿物,应想到本病的可能,以手术为主的治疗能改善预后。     

Systemic treatment of pancreatic neuroendocrine tumors

Pancreatic neuroendocrine tumours (pNET) are a group of heterogeneous tumours. Despite being rare, their incidence is rising. While localized lesions can be cured with surgical resection, most patients present with metastatic disease. Systemic therapy is thus integral for the management of this rare entity. Numerous advancements have been made in the past decade in the systemic treatment of unresectable, metastatic pNET. Treatment options for pNET include somatostatin analogues, cytotoxic chemotherapy, targeted agents and peptide receptor radionuclide therapy. In this Review, we present the latest evidence for these treatment options.     

无功能性胰腺神经内分泌肿瘤的CT诊断

目的探讨无功能性胰腺神经内分泌肿瘤(NF-PNETs)的CT表现及诊断价值。方法对9例经病理学证实的NF-PNETs的CT及临床资料进行回顾性分析。结果 9例均为单发,位于胰腺头部4例,体、颈部3例,尾部2例;4例呈类圆形,5例分叶状。肿瘤最大直径约27~109 mm,平均76 mm,边界相对较清。4例实性、5例囊实性,CT平扫实性部分呈不均匀等及较低混杂密度,囊变、坏死区为低密度。增强扫描实性成分多呈明显或较明显强化,以动脉期明显。肿瘤边缘可见完整或不完整环形包膜,增强较明显,延迟强化。4例伴肝脏转移,3例腹膜后淋巴结转移并周边广泛侵犯,4例伴胆总管和/或主胰管稍增宽。结论 NF-PNETs以单发较大肿块常见,CT对其诊断及鉴别有较大价值,平扫多数呈较低密度,边界相对较清晰,增强早期较明显强化并见包膜延迟强化,容易血行转移但胰管扩张相对较少见。     

血清嗜铬粒蛋白A水平与无功能性胰腺神经内分泌瘤肝转移的关系

目的 探讨血清嗜铬粒蛋白A(CgA)水平与无功能性胰腺神经内分泌瘤(pNETs)肝转移肿瘤负荷及患者预后的关系.方法 回顾性分析2011年12月至2013年12月复旦大学附属中山医院收治的64例无功能性pNETs肝转移患者的临床资料.39例患者具有临床和随访资料,25例患者仅有临床资料.64例患者中,58例具有尚未发生肝转移时的血清CgA水平资料,51例具有已发生肝转移时的血清CgA水平资料.采用门诊方式进行随访,患者出院后每3～6个月定期复查实验室和影像学检查.患者血清CgA水平升高或降低程度≥50％治疗肝转移瘤前的血清CgA水平,定义为血清CgA水平升高或降低,其余定义为血清CgA水平稳定.随访时间截至2014年3月.采用ELISA法检测血清CgA.CT检查三维重建测定肝转移肿瘤负荷.采用国际实体瘤疗效评价标准评价无功能性pNETs肝转移患者疗效.将完全缓解和部分缓解归为治疗缓解,疾病稳定归为疾病稳定,疾病进展和肿瘤复发归为疾病进展.计量资料用M(P25,P75)表示,计数资料检验采用Fisher确切概率法,计量资料多组间比较采用Kruskal-Wallis检验,两两比较采用Mann-Whitney检验,采用Spearman等级相关分析进行相关性检验.结果 无功能性pNETs肝转移患者血清CgA水平为94.8 μg/L(67.4 μg/L,181.4 μg/L),显著高于尚未发生肝转移时的血清CgA水平59.9 μg/L(39.1μg/L,88.5 μg/L),两者比较,差异有统计学意义(Z=-3.96,P＜0.05).无功能性pNETs肝转移患者血清CgA水平与无功能性pNETs肝转移肿瘤负荷呈正相关(r=0.486,P＜0.05).39例具有临床和随访资料的患者中35例获得完整随访,中位随访时间为10个月.14例治疗缓解的患者中,血清CgA水平降低和稳定患者比例分别为11/14和3/14;12例疾病稳定的患者中,血清CgA水平降低、稳定和升高的患者比例分别为4/12、7/12和1/12;9例疾病进展的患者中,血清CgA水平稳定和升高患者比例分别为2/9和7/9.3种不同疗效的患者血清CgA水平降低和升高患者比例比较,差异均有统计学意义(F =11.02,18.82,P＜0.05);血清CgA水平稳定患者比例比较,差异无统计学意义(F=5.68,P＞0.05).治疗缓解的患者血清CgA水平降低患者比例显著高于其余两种疗效的患者,差异有统计学意义(P＜0.05);疾病进展的患者血清CgA水平升高患者比例显著高于其余两种疗效的患者,差异有统计学意义(P＜0.05).结论 高血清CgA水平可能提示无功能性pNETs患者发生肝转移,疗效不佳.血清CgA水平越高,可能提示肝转移肿瘤负荷越大.     

Clinicopathological features and long-term prognosis of purely cystic pancreatic neuroendocrine tumors: A single-center experience

BackgroundPancreatic neuroendocrine tumors (pNETs) are heterogenous neoplasms, of which the prognosis varies widely. Purely cystic pancreatic neuroendocrine tumors (C-pNETs) are a small subset of pNETs in which data are extremely rare. This study aimed to compare clinicopathological and long-term survival differences between C-pNETs and solid pNETs (S-pNETs).MethodsA retrospective review of 242 patients with pNETs underwent resection in our institution from 2009 to 2019 was conducted. Demography characteristics, clinicopathological features and long-term outcomes of them were analyzed.ResultsSixteen out of 242 patients (6.6%) were identified as C-pNETs. Compared with S-pNETs, C-pNETs were more frequently non-functional (75% vs 45%, P = 0.02), and the median tumor diameter of C-pNETs was smaller (36 mm vs. 47 mm, P = 0.001). And the accuracy of preoperative diagnosis of C-pNETs was significantly lower (31% vs 78%, P = 0.001). Of note, the majority of C-pNETs were well-differentiated with G1 (81% vs 35%, P = 0.001). And there were no G3 (0 vs 7%, P = 0.001) in C-pNETs. No T4 stage or R1/R2 surgical margin detected in C-pNETs. And only one C-pNETs (6%) had regional lymph node metastasis (N) or synchronous distant metastasis (M). Additionally, only one patient with C-pNETs (6%) suffered tumor recurrence, compared with 24 (13%) for S-pNETs. And survival analysis showed the patients with C-pNETs seemed to be with better disease-free survival (P = 0.26).ConclusionC-pNETs are rare subtype with possibly less aggressive behavior comparing with their solid counterparts. Recurrence and tumor-related death still occurs in patients with resected C-pNETs, although they tend to be with more favorable prognosis.     

Clinical presentation and outcome of nonfunctional pancreatic neuroendocrine tumors in a modern cohort

Background

The natural history of nonfunctional pancreatic neuroendocrine tumors (NF-PNETs) is largely unstudied due to its rarity. The primary goal of this study was to characterize clinical features and outcomes of incidental NF-PNETs.

Methods

An institutional review board–approved retrospective study of patients with NF-PNET evaluated by the Surgical Oncology of University of Nebraska Medical Center was performed. Patients were evaluated with dedicated pancreatic and liver imaging using multiphasic computed tomographic scan and dedicated magnetic resonance imaging protocols.

Results

Forty-six patients (male, 47.8%) were evaluated, and 35 ultimately resected. Of these, 16 tumors were discovered incidentally. The median age was 62 and 59 years in incidental and symptomatically discovered, respectively. Incidental median size was 2.4 cm vs 6 cm in the symptomatic group, with a P value of .037. The presence of lymphatic and liver metastases was 10% and 25% incidental and 45% and 67% for those with symptoms (lymphatic involvement, P = .05; liver metastases P = .07). Median overall survival was 45 and 76 months (P = .03).

Conclusions

Incidentally discovered NF-PNETs represent a malignancy with more questions than answers. Our series indicates that these cancers are more indolent than previously believed.     

Cystic pancreatic neuroendocrine tumors: Is preoperative diagnosis possible?

Pancreatic neuroendocrine tumors rarely undergo cystic degeneration leading to a radiologic appearance, which is often interpreted as a pancreatic mucinous cystadenoma or pseudocyst. We reviewed our experience with 38 neuroendocrine tumors, four of which were cystic, and 24 other cystic pancreatic tumors (mucinous cystadenoma [n = 5], cystadenocarcinoma [n = 6], serous cystadenoma [n = 3], solid/cystic papillary neoplasm [n = 3], intraductal papillary mucinous tumor [n = 6], and mucinous adenocarcinoma [n = 1]) managed operatively between 1990 and 2000. This review was undertaken to identify clinical and pathologic features useful for preoperative diagnosis of cystic neuroendocrine tumors. Two of the four patients with cystic neuroendocrine tumors presented with abdominal pain, one patient was asymptomatic, and one patient had hypoglycemia. Three of the four cystic neuroendocrine tumors were identified by CT scan, and none were biopsied preoperatively. Preoperative diagnoses included mucinous cystadenoma in two patients (n = 2), pancreatic cystic neoplasm in one patient, (n = 1) and insulinoma in one patient (n = 1). All four cystic neuroendocrine tumors were benign and were completely resected (distal pancreatectomy [n = 2], enucleation [n = 2]). Cystic neuroendocrine tumors are difficult to diagnose preoperatively because the majority of these tumors are nonfunctional, and CT does not differentiate these tumors from other cystic neoplasms. Cystic neuroendocrine tumors represent a subgroup of pancreatic cystic and neuroendocrine tumors with malignant potential. Their high resectability rate further supports the role of surgical exploration and resection in the treatment of pancreatic cystic neoplasms. Presented at the Third Americas Hepatopancreatobiliary Congress, Miami, Fla., February 22–25, 2001.     

胰腺神经内分泌肿瘤患者腹腔镜术后住院时间延长的危险因素分析

目的 探究胰腺神经内分泌肿瘤（pNETs）患者行腹腔镜手术后住院时间延长的危险因素。方法 回顾性分析2015年1月至2022年8月在浙江省人民医院行腹腔镜手术治疗的91例pNETs患者的临床资料。以术后住院时间是否超过中位住院时间12 d将患者分为住院时间正常组（NLOS组,≤12 d,n=50）和住院时间延长组（PLOS组,>12 d,n=41）。通过单因素和多因素Logistic回归分析患者术后住院时间延长的危险因素,用AUC和Brier分数评价区分度和校准度。结果 单因素及多因素分析显示,手术时间>210 min(OR=8.850,95%CI 2.699～29.013,P<0.001)和腹腔镜手术方式[腹腔镜胰十二指肠切除术（LPD）:OR=5.753,95%CI 1.062～31.155,P=0.042;腹腔镜胰腺体尾切除术（LDP:OR=8.547,95%CI1.908～38.291,P=0.005]是患者住院时间延长的独立危险因素。此时相应的AUC为0.785,Brier分数为0.182。结论 手术时间和手术方式是预测pNETs患者腹腔镜术后住院时间是否延...