2001~2010年中国胰腺神经内分泌肿瘤的临床流行病学特征分析

[目的]分析中国2001～2010年胰腺神经内分泌肿瘤患者的流行病学特征.[方法]将中国大陆按传统的习惯分为七个区,每个大区选取至少一家肿瘤医院和综合医院.收集医院2001～2010年间所有经病理确诊为胰腺神经内分泌肿瘤的病例信息,摘录至病例信息收集表并进行统计分析.[结果]研究共纳入中国七个地理大区23家医院共633例胰腺神经内分泌肿瘤患者,平均年龄45.7±14.0岁,男性占41.2％.10年间病例数持续增加,且以低级别早期肿瘤的增加为主.功能性胰腺神经内分泌肿瘤占57.1％.其中,出现Whipple三联征的患者占46.8％.诊断时局限于胰腺内(70.0％)、G1/G2级(57.8％)的患者较多.临床上最常用的影像学检查手段为超声(78.2％)和CT(85.9％).手术是最主要的治疗方式,92.4％的患者接受了手术切除.[结论]中国胰腺神经内分泌肿瘤患者的诊断年龄较早,分期及分级较低,且10年间以低级别病变的检出增加为主.传统影像学手段对于肿瘤的检出率较高,手术治疗为主要的治疗方式.     

胃肠胰腺神经内分泌肿瘤国际诊断共识的解读

近年来胃肠胰腺神经内分泌肿瘤的发病率不断上升,随着对疾病认识的不断深入,现在从疾病术语、诊断到治疗规范都发生了巨大的变化。本文结合世界卫生组织(WHO)2010版消化系统肿瘤病理分类、欧洲神经内分泌肿瘤协会(ENETs)共识、北美神经内分泌肿瘤协会(NANETs)共识和美国国立癌症网络(NCCN)指南,对胃肠胰腺神经内分泌肿瘤诊断进行解读。     

晚期胃肠胰腺神经内分泌肿瘤化疗进展

正胃肠胰腺神经内分泌肿瘤(gastroenteropancreatic neuroendocrine tumor,GEP-NET)是一类罕见的起源于胃肠胰腺神经内分泌细胞的肿瘤,具有特殊的临床和生物学特征。其发病约占所有消化道肿瘤的2%[1],但近年发病率增长迅速[2]。有功能的NET常表现为过量分泌肿瘤相关物质引起相应症     

结直肠神经内分泌肿瘤的研究进展

结直肠神经内分泌肿瘤来源于结直肠神经内分泌细胞,发病率较低,但近年有上升趋势.WHO病理学将结直肠神经内分泌肿瘤分为神经内分泌瘤、神经内分泌癌、混合型腺-神经内分泌癌和增生性及肿瘤前病变.结直肠神经内分泌肿瘤细胞具有激素合成及分泌功能,但不一定都出现相应的临床症状.不同分类、分期的结直肠神经内分泌肿瘤的诊断及治疗方法也...     

胸腺神经内分泌肿瘤临床病理学亚型和诊治进展

胸腺神经内分泌肿瘤（neuroendocrine tumors of the thymus,NETT）是一种临床上罕见的位于前上纵隔的胸腺恶性肿瘤。它是一种主要或完全由神经内分泌细胞构成的上皮性肿瘤,需与含有散在的和成团的神经内分泌细胞的胸腺癌相鉴别。该病临床表现复杂多变且具有高度侵袭性。文章重点对NETT临床病理学亚型和诊治进展作一综述。     

71例胰腺神经内分泌肿瘤诊治分析

目的探讨经病理证实的胰腺神经内分泌肿瘤预后及其相关因素。方法收集1999年1月至2014年7月间收治的71例胰腺神经内分泌肿瘤的临床资料,进行随访及统计学分析。结果非功能性肿瘤65例,主要表现为腹痛、腹胀、腹部肿块等,其中23例无明显症状;功能性肿瘤6例,以内分泌紊乱症状为特点,4例以低血糖反应为主要表现。71例患者中,65例接受手术治疗;另6例保守治疗。随访时间为1~184个月,中位随访时间为41个月。45例存活,其中40例未见肿瘤复发,5例出现远处转移,16例死亡,7例失访。单因素分析结果显示,手术方式(2=8.433)、肿瘤分级(2=7.685)、肿瘤分期(2=15.204)、有无淋巴结转移(2=8.508)、有无神经侵犯(2=14.531)和有无远处转移(2=1.060)与预后相关。多因素分析结果显示,有无神经侵犯是影响胰腺神经内分泌肿瘤预后的独立因素。结论手术方式、肿瘤分级、肿瘤分期、淋巴结转移情况、神经侵犯情况和有无远处转移均可影响胰腺神经内分泌肿瘤的预后,有神经侵犯则提示预后不良。     

胰腺神经内分泌肿瘤的外科治疗进展

<正>神经内分泌肿瘤(neuroendocrine neoplasm,NEN)是一种罕见的异质性肿瘤[1],一般起源于神经内分泌细胞,占所有神经内分泌肿瘤的1%～2%[2],对人体健康有很大危害。这种肿瘤可出现在人体任何部位,且具有很高的转移倾向。这类肿瘤早在1907年就被命名为"类癌",其在发展过程中表现出明显的生物学特征,如惰性、低度恶性和转移性等[3]。相关研究结果表明,胰腺神经内分泌肿瘤(pancreatic neuroendocrine tumors,pNENs)约占神经内分泌肿瘤的     

胰腺神经内分泌肿瘤的研究进展

胰腺神经内分泌瘤是起源于胰腺内分泌部的罕见肿瘤,发病率低,且有恶变倾向,早期诊断和治疗是提高胰腺神经内分泌瘤患者生存的关键。本文结合新的基础研究及临床试验,对该病的分类、临床表现、临床诊断和治疗方式的研究进展进行综述。     

17例无功能性胰腺神经内分泌肿瘤的诊治分析

目的:报道17例无功能性胰腺神经内分泌肿瘤并分析其诊治方法。方法:回顾性分析自2002年1月至2014年8月我院收治的17例无功能性胰腺神经内分泌肿瘤患者的临床资料并结合文献进行复习讨论。结果:肿瘤位于胰头部8例,胰体尾部8例,弥漫整个胰腺1例。17例患者均行手术治疗,术后病理检查均提示无功能性胰腺神经内分泌肿瘤。瘤体直径3-15cm,均呈浸润性生长,3例发生肝转移。1例术后3小时因肺梗塞死亡,另随访到13例,9例存活至今,2例死于肿瘤复发,各有1例死于肺部感染及术后肝转移。结论:无功能性胰腺神经内分泌肿瘤确诊需依赖病理学检查,以手术为主的综合治疗能够提高患者生存率,改善预后。     

127例支气管肺神经内分泌肿瘤的特征及预后分析

目的:支气管肺神经内分泌肿瘤包括典型类癌、不典型类癌、大细胞神经内分泌癌和小细胞肺癌.尽管拥有类似的组化染色特征,但其临床特点和预后情况并不一致.本研究拟观察肺神经内分泌肿瘤的临床、病理特征并分析影响其预后的因素.方法:回顾性分析天津医科大学附属肿瘤医院1975年4月至2009年7月收治的127例肺神经内分泌肿瘤临床资料,分别对性别、年龄、吸烟史、肿瘤家族史、组织学类型、TNM分期及治疗等项目对预后的影响进行了单因素及多因素预后分析.结果:全组患者的1、3及5年生存率分别为80.2%、46.9%和40.6%.典型类癌、不典型类癌、小细胞肺癌及大细胞神经内分泌癌的5年生存率分别为79.3%、41.8%、11.4%和0.单因素分析显示,肿瘤大小(P=0.000)、组织学类型(P=0.000)、TNM分期(P=0.000)和有无术后辅助放、化疗(P=0.000)是影响预后的因素.多因素分析显示,TNM分期(P=0.040)及有无术后辅助放、化疗(P=0.032)是影响预后的独立因素.结论:根治性手术是肺神经内分泌肿瘤主要治疗手段.影响其预后的独立因素是TNM分期和有无术后辅助放化疗.     

Survivals of patients with surgically treated and High-grade pancreatic neuroendocrine carcinomas: A comparative study between two American Joint Committee on Cancer 8th tumor-node-metastasis staging systems

Background and objectiveWe aimed to compare the two new defined tumor-node-metastasis (TNM) systems in the American Joint Committee on Cancer (AJCC) 8th staging manual for overall survival (OS) analysis of G3 pancreatic neuroendocrine carcinomas (p-NECs) that are currently proposed for pancreatic exocrine adenocarcinomas (p-EACs) and G1/G2 pancreatic neuroendocrine tumors (p-NETs), respectively.MethodsThe data of patients who were surgically treated and histopathologically diagnosed with G3 p-NECs at West China Hospital of Sichuan University from January 2002 to June 2017 were retrospectively analyzed and compared using the two new AJCC staging systems.ResultsApplying the p-EAC AJCC 8th TNM staging system to G3 p-NECs, the estimated 3-year OSs for each stage were 86.7%, 76.0%, 44.5% and 20.7%, respectively (P < 0.001). According to the G1/G2 p-NETs staging system, the estimated OSs at 3 years for each new AJCC stage were 100.0%, 83.6%, 47.1% and 20.7%, respectively (P < 0.001). The system for p-EACs significantly discriminated the survival difference of G3 p-NECs between Stage I and Stage II (P = 0.019), while the other one for G1/G2 p-NETs could not (P = 0.108). The consistent results of Akaike information criteria with Harrell's concordance index indicated that the AJCC 8th staging system for p-EACs was superior when applied to G3 p-NECs for its better prognostic stratification and more accurate prediction ability for OS.ConclusionsOur analysis demonstrated that both TNM systems in the AJCC 8th staging manual were prognostic for patients with G3 p-NECs; however, the classification originally applied to p-EACs was superior and supported its use in clinical practice.     

Revisiting the Prognostic Heterogeneity of AJCC Stage IV Carcinomas of the Upper Urinary Tract

Background

Current staging paradigms from the American Joint Committee on Cancer (AJCC) staging system for upper urinary tract carcinomas treat locoregionally advanced (T4 and N+) and metastatic (M1) patients as a single entity (stage IV). The current study proposes a modification of the AJCC staging system where these 2 entities are separated.

基于肿瘤位置的改良分期系统对T1期胰腺导管腺癌预后的价值

目的:探究基于肿瘤位置的改良分期系统对T1期胰腺导管腺癌预后的价值。方法:收集南阳市第一人民医院18年间进行胰腺癌手术的患者共1 873例,其中1 291例患者的肿瘤位置在胰头,582例患者的肿瘤位置在胰体/尾部。比较不同分组患者的肿瘤特异性生存率和肿瘤术后的预后危险因素。结果:在T1期患者中,胰头肿瘤患者的预后较胰尾肿瘤的预后差。多因素分析结果显示,胰头位置、肿瘤大小、N分期是T1期胰腺导管腺癌患者更低存活率的独立危险因素（危险比为1.49,95%可信区间为1.13~1.87,P=0.022）。基于肿瘤位置的改良分期系统较第八版AJCC分期系统更能区分早期胰腺导管腺癌的不同分组。结论:肿瘤位置是可切除胰腺导管腺癌患者预后的预测因子,基于肿瘤位置的改良T1亚分期对胰腺导管腺癌的预后预测准确有效。     

The ENETS guidelines: the new TNM classification system

The WHO definition of the 3 classes of well differentiated endocrine tumor, well differentiated endocrine carcinoma and poorly differentiated carcinoma, allows an efficient general classification of GEP NETs fitting European Union (EU) current clinical use. The ENETS grading and TNM staging systems are complementary tools allowing to improve patients' stratification, and their adoption is strongly recommended. However, the prognostic value of these tools is still under investigation and more data are needed to support their usefulness at all gut sites and in perspective series. Similarly, the four classes system adopted by the WHO for the endocrine tumors of the lung allows a prognostically effective stratification of patients. A common grading system is advocated for lung and GEP NETS.     

Sporadically occurring functional pancreatic endocrine tumors: review of recent literature

PURPOSE OF REVIEW: Pancreatic neuroendocrine tumors are rare neoplasms often associated with a clinical syndrome. Their rarity makes a comprehensive study difficult at any single institution, while their uniqueness makes them desirable for investigation. This review summarizes recent information and advancements concerning pancreatic neuroendocrine tumor diagnosis, imaging characteristics, treatment algorithms, and staging. RECENT FINDINGS: Insulinomas and gastrinomas comprise the majority of functional pancreatic neuroendocrine tumors. Advances in their identification and diagnostic evaluation, imaging techniques, and treatment algorithms are presented. Furthermore, a new staging classification system has been proposed which may significantly improve the ability to conduct future multi-institutional investigations on pancreatic neuroendocrine tumors. SUMMARY: Although rare, a thorough understanding of pancreatic neuroendocrine tumors is essential for all physicians due to the wide variety of symptoms with which patients present. Currently, patients are often misdiagnosed for extended periods of time. This review summarizes the recently published literature about diagnosis, imaging, treatment, and staging of pancreatic neuroendocrine tumors.     

External validation of a modified 8th AJCC TNM system for advanced gastric cancer: Long-term results in southern China

Background

The 8th edition of AJCC TNM staging manual for gastric cancer (GC) has been validated by several studies. A modified staging system based on it and total harvested number of lymph nodes (LNs; cutoff: 30) is suggested to improve predictive capacities for advanced GC. This study is designed to validate the modified method using a single-center database in Southern China.

Validation of a postresection pancreatic adenocarcinoma nomogram for disease-specific survival.

PURPOSE: Nomograms are statistically based tools that provide the overall probability of a specific outcome. They have shown better individual discrimination than the current TNM staging system in numerous patient tumor models. The pancreatic nomogram combines individual clinicopathologic and operative data to predict disease-specific survival at 1, 2, and 3 years from initial resection. A single US institution database was used to test the validity of the pancreatic adenocarcinoma nomogram established at Memorial Sloan-Kettering Cancer Center. PATIENTS AND METHODS: The nomogram was created from a prospective pancreatic adenocarcinoma database that included 555 consecutive patients between October 1983 and April 2000. The nomogram was validated by an external patient cohort from a retrospective pancreatic adenocarcinoma database at Massachusetts General Hospital that included 424 consecutive patients between January 1985 and December 2003. RESULTS: Of the 424 patients, 375 had all variables documented. At last follow-up, 99 patients were alive, with a median follow-up time of 27 months (range, 2 to 151 months). The 1-, 2-, and 3-year disease-specific survival rates were 68% (95% CI, 63% to 72%), 39% (95% CI, 34% to 44%), and 27% (95% CI, 23% to 32%), respectively. The nomogram concordance index was 0.62 compared with 0.59 with the American Joint Committee on Cancer (AJCC) stage (P = .004). This suggests that the nomogram discriminates disease-specific survival better than the AJCC staging system. CONCLUSION: The pancreatic cancer nomogram provides more accurate survival predictions than the AJCC staging system when applied to an external patient cohort. The nomogram may aid in more accurately counseling patients and in better stratifying patients for clinical trials and molecular tumor analysis.     

Review of the 2001 AJCC staging system for cutaneous malignant melanoma

The American Joint Committee on Cancer (AJCC) staging system for melanoma has recently been revised and published. The previous staging system had not been substantially modified since the late 1980s. In a series of papers, the staging system for melanoma was critically analyzed, and many shortcomings were identified. Many well-established prognostic factors were not used in the staging system. This assessment has led to a substantially modified staging system for cutaneous melanoma in 2001 that is a considerable improvement over past staging systems, albeit more complex. The following modifications are the most important: 1) The primary determinant of tumor (T) staging is tumor thickness as measured in millimeters. The Clark level of invasion is now used only for defining T1 (< or = 1mm) melanomas; 2) The cutpoints for tumor thickness are less than or equal to 1 mm, 1 to 2 mm, 2 to 4 mm, and greater than 4 mm; 3) Ulceration has been added in describing the primary tumor; 4) Local recurrence, satellite disease, and in-transit metastases have similar prognosis and are now all classified together as regional stage III disease; 5) Size of lymph node as prognostic factor has been eliminated and replaced with the number of positive nodes; 6) The presence of an elevated serum lactic dehyrogenase level is used in the metastasis (M) category. This revised staging system more precisely defines prognosis and will improve the stratification of patients in future clinical trials.     

Survival outcomes used to validate version 9 of the American Joint Committee on Cancer staging system for appendiceal cancer

The standard for cancer staging in the United States for all cancer sites, including primary carcinomas of the appendix, is the American Joint Committee on Cancer (AJCC) staging system. AJCC staging criteria undergo periodic revisions, led by a panel of site-specific experts, to maintain contemporary staging definitions through the evaluation of new evidence. Since its last revision, the AJCC has restructured its processes to include prospectively collected data because large data sets have become increasingly robust and available over time. Thus survival analyses using AJCC eighth edition staging criteria were used to inform stage group revisions in the version 9 AJCC staging system, including appendiceal cancer. Although the current AJCC staging definitions were maintained for appendiceal cancer, incorporating survival analysis into the version 9 staging system provided unique insight into the clinical challenges in staging rare malignancies. This article highlights the critical clinical components of the now published version 9 AJCC staging system for appendix cancer, which (1) justified the separation of three different histologies (non-mucinous, mucinous, signet-ring cell) in terms of prognostic variance, (2) demonstrated the clinical implications and challenges in staging heterogeneous and rare tumors, and (3) emphasized the influence of data limitations on survival analysis for low-grade appendiceal mucinous neoplasms.