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.     

对比不同影像学方法对胰腺神经内分泌肿瘤的检出率

目的探讨不同影像学检查方法对检出胰腺神经内分泌肿瘤(P-NEN)的价值。方法回顾性分析40例(共43个病灶)经手术后病理证实的P-NEN患者的影像学资料,包括CT、MRI、超声内镜(EUS)、PET/CT,与手术结果相对照,分析各种影像学方法对P-NEN的检出率。结果 CT、MRI、EUS及PET/CT对P-NEN病灶的检出率分别为76.74%(33/43)、94.87%(37/39)、94.59%(35/37)和51.52%(17/33)。MRI与EUS病灶检出率差异无统计学意义(P均0.05),余两两比较差异均有统计学意义(P均0.05)。结论 MRI可作为P-NEN的首选影像学检查方法。     

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.     

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。     

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.     

Therapeutic strategies for gastroenteropancreatic neuroendocrine neoplasms: State-of-the-art and future perspectives

Although gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) have always been considered rare tumors, their incidence has risen over the past few decades. They represent a highly heterogeneous group of neoplasms with several prognostic factors, including disease stage, proliferative index (Ki67), and tumor differentiation. Most of these neoplasms express somatostatin receptors on the cell surface, a feature that has important implications in terms of prognosis, diagnosis, and therapy. Although International Guidelines propose algorithms aimed at guiding therapeutic strategies, GEP-NEN patients are still very different from one another, and the need for personalized treatment continues to increase. Radical surgery is always the best option when feasible; however, up to 80% of cases are metastatic upon diagnosis. Regarding medical treatments, as GEP-NENs are characterized by relatively long overall survival, multiple therapy lines are adopted during the lifetime of these patients, but the optimum sequence to be followed has never been clearly defined. Furthermore, although new molecular markers aimed at predicting the response to therapy, as well as prognostic scores, are currently being studied, their application is still far from being part of daily clinical practice. As they represent a complex disease, with therapeutic protocols that are not completely standardized, GEP-NENs require a multidisciplinary approach. This review will provide an overview of the available therapeutic options for GEP-NENs and attempts to clarify the possible approaches for the management of these patients and to discuss future perspectives in this field.     

胰腺神经内分泌肿瘤外科治疗策略

胰腺神经内分泌肿瘤是一类起源于胰腺神经内分泌细胞,具有较高异质性的潜在恶性肿瘤,有较好的生物学行为和预后。外科手术治疗被认为是最重要且唯一可治愈胰腺神经内分泌肿瘤的方式,但治疗策略上仍存在诸多争议,不但要充分评估肿瘤性质、体积、位置、数量、有无周围器官及血管侵犯、有无淋巴结及远处转移等,同时还要综合考虑胰腺手术的风险和患者的自身条件。现就近年来胰腺神经内分泌肿瘤的外科治疗进展做一述评。     

无功能性胰腺神经内分泌肿瘤的MRI表现及治疗

[摘要] 目的 探讨无功能性胰腺神经内分泌肿瘤（non-Punctioning pancreatic neuroendocrine neoplasms,NF-pNENs）的MRI表现及治疗措施,提高诊疗水平。方法 回顾性分析11例经病理学证实的无功能性胰腺神经内分泌肿瘤的MRI及临床资料。结果 11例肿瘤均为单发,7例呈类圆形,4例呈不规则分叶状。7例边界较清晰,4例局部侵犯导致边界不清。肿瘤最大直径约23～137 mm,平均60.1 mm。其中实性5例,囊实性5例,囊性1例。MRI平扫肿瘤实性成分呈不均匀T1WI较低T2WI较高信号,囊变、坏死区为T1WI低T2WI高信号,DWI均为不均匀高信号。增强实性部分7例明显持续强化,3例呈中等持续强化,1例轻度延迟强化。9例见完整或不完整的包膜结构较明显强化,3例见胆总管和（或）主胰管增宽,3例肝脏转移,2例腹膜后淋巴结转移,2例腹腔少量积液。11例均经手术治疗,9例达到根治性切除。术后7例接受全身化疗,4例仅接受中医药治疗。术后随访6～63个月,3例死亡,平均生存时间35.7个月;8例仍存活。结论 MRI有利于反映NF-pNENs的病理学变化,对其诊断具有重要价值。手术切除是NF-pNENs的主要治 疗手段,一旦确诊应争取根治性切除。     

Resection of pancreatic neuroendocrine tumors: results of 70 cases

HYPOTHESIS: Neuroendocrine tumors of the pancreas can be managed surgically with excellent outcomes. DESIGN: Retrospective case series. SETTING: Academic medical center. PATIENTS: Seventy consecutive patients who underwent resection for pancreatic neuroendocrine tumors between January 1, 1990, and December 31, 2005. INTERVENTIONS: Pancreaticoduodenectomy, distal pancreatectomy, or enucleation. MAIN OUTCOME MEASURES: Postoperative morbidity, mortality, and long-term survival. RESULTS: Of the 70 patients, 50 (71.4%) had nonfunctional tumors. Thirty-seven patients (52.9%) had neuroendocrine carcinomas and 13 (18.6%) had benign islet cell neoplasms. Twenty patients had functional tumors. Of these 20 patients, 16 had insulinomas, 2 had glucagonomas, and 2 had gastrinomas. Twenty-seven patients underwent pancreaticoduodenectomy, 32 had distal pancreatectomy, and 11 underwent enucleation. Patients undergoing enucleation as compared with those not undergoing enucleation were younger (mean age, 39 vs 51 years, respectively; P = .009) and had smaller tumors (mean tumor size, 2 vs 5 cm, respectively; P<.001). Postoperative complications occurred in 13 patients (48.1%) after pancreaticoduodenectomy, in 4 patients (12.5%) after distal pancreatectomy, and in 0 patients after enucleation. There were no perioperative mortalities. With a median follow-up of 50 months, the 5-year actuarial survival for the patients with malignant neuroendocrine carcinomas (n = 37) was 77%, and all of the patients with functional tumors are alive. The presence of lymphovascular invasion closely approached significance when survival was evaluated (P = .06). Lymph node status, perineural invasion, and liver metastasis did not impact survival. CONCLUSIONS: This single-institutional case series demonstrates that pancreatic neuroendocrine tumors can be safely resected without mortality and with minimal morbidity. The presence of lymphovascular invasion can be used to classify neuroendocrine tumors as malignant, and this appears to predict survival. Patients with malignant tumors can expect long-term survival even in the setting of metastatic disease.     

胰腺神经内分泌肿瘤诊疗现状

神经内分泌肿瘤（NEN）是一种罕见的高异质性肿瘤,起源于肽能神经元和神经内分泌细胞,可发生在支气管、肺部和胃肠、胰腺等多种部位,我国以胰腺神经内分泌肿瘤（pNEN）最为多见,近几十年来发病率显著上升。笔者主要从pNEN的分级标准、诊断、治疗等方面对该疾病的最新诊疗研究现状进行介绍,以期为临床诊疗提供指导和参考。     

Endoscopic ultrasound guided radiofrequency ablation,for pancreatic cystic neoplasms and neuroendocrine tumors

AIM: To outline the feasibility, safety, adverse events and early results of endoscopic ultrasound (EUS)-radiofrequency ablation (RFA) in pancreatic neoplasms using a novel probe.METHODS: This is a multi-center, pilot safety feasibility study. The intervention described was radiofrequency ablation (RF) which was applied with an innovative monopolar RF probe (1.2 mm Habib EUS-RFA catheter) placed through a 19 or 22 gauge fine needle aspiration (FNA) needle once FNA was performed in patients with a tumor in the head of the pancreas. The Habib™ EUS-RFA is a 1 Fr wire (0.33 mm, 0.013”) with a working length of 190 cm, which can be inserted through the biopsy channel of an echoendoscope. RF power is applied to the electrode at the end of the wire to coagulate tissue in the liver and pancreas.RESULTS: Eight patients [median age of 65 (range 27-82) years; 7 female and 1 male] were recruited in a prospective multicenter trial. Six had a pancreatic cystic neoplasm (four a mucinous cyst, one had intraductal papillary mucinous neoplasm and one a microcystic adenoma) and two had a neuroendocrine tumors (NET) in the head of pancreas. The mean size of the cystic neoplasm and NET were 36.5 mm (SD ± 17.9 mm) and 27.5 mm (SD ± 17.7 mm) respectively. The EUS-RFA was successfully completed in all cases. Among the 6 patients with a cystic neoplasm, post procedure imaging in 3-6 mo showed complete resolution of the cysts in 2 cases, whilst in three more there was a 48.4% reduction [mean pre RF 38.8 mm (SD ± 21.7 mm) vs mean post RF 20 mm (SD ± 17.1 mm)] in size. In regards to the NET patients, there was a change in vascularity and central necrosis after EUS-RFA. No major complications were observed within 48 h of the procedure. Two patients had mild abdominal pain that resolved within 3 d.CONCLUSION: EUS-RFA of pancreatic neoplasms with a novel monopolar RF probe was well tolerated in all cases. Our preliminary data suggest that the procedure is straightforward and safe. The response ranged from complete resolution to a 50% reduction in size.     

Long-term results of surgery for pancreatic neuroendocrine neoplasms in patients with MEN1

Purpose  

This study was made to evaluate long-term results of an aggressive surgical approach for pancreaticoduodenal neuroendocrine neoplasms (pNENs) in patients with multiple endocrine neoplasia type 1 (MEN1).     

非功能性胰腺神经内分泌肿瘤的MRI检查特征

胰腺神经内分泌肿瘤(pNENs)是一组具有潜在恶性的少见肿瘤,依据临床有无内分泌症状分为功能性和非功能性两类.MRI检查有助于非功能性pNENs的术前诊断.本研究回顾性分析2010年3月至2014年2月解放军总医院收治的22例非功能性pNENs患者的临床资料.其结果显示:MRI动态增强扫描示肿瘤呈持续或渐进性中度至明显强化、肿瘤周边包膜中度至明显强化是非功能性pNENs的特征性表现,突破包膜生长或明显分叶样生长改变提示肿瘤可能具有局部侵袭性生长趋势.该特征对该类肿瘤的诊断与鉴别诊断具有重要价值.     

Peptide receptor radionuclide therapy as neoadjuvant therapy for resectable or potentially resectable pancreatic neuroendocrine neoplasms

Background

Peptide receptor radionuclide therapy is a valid therapeutic option for pancreatic neuroendocrine neoplasms. The aim of this study was to describe an initial experience with the use of peptide receptor radionuclide therapy as a neoadjuvant agent for resectable or potentially resectable pancreatic neuroendocrine neoplasms.

Cystic pancreatic neuroendocrine neoplasms with uncertain malignant potential: Report of two cases

Neuroendocrine tumors of the pancreas (NETP) represent only 1%–2% of all pancreatic neoplasms. They can be classified as functioning or non-functioning, respectively, according to the presence or absence of paraneoplastic syndrome. Case 1 concerned a 70-year-old woman with a cystic lesion of the pancreatic head and body. All tumor markers were negative. The patient underwent a distal pancreatectomy. The histology revealed a well-differentiated endocrine tumor with uncertain malignant potential. Case 2 was a 61-year-old man with chronic polyserositis. The serum tumor markers were negative, while he was strongly positive for intracystic tumor markers carcinoembryonic antigen, carbohydrate antigen (CA) 19–9, and CA 125. The patient underwent a cephalo-pancreatic duodenectomy. The preoperative differential diagnosis of cystic NETP is still a challenge due to the high rate of the nonfunctional variant. Although cystic NETPs are well differentiated, they are still tumors with a malignant potential, and therefore an early diagnosis and radical surgical resection could be associated with a better long-term survival.