2019WHO消化系统肿瘤分类 第五版 大纲、前言翻译
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大纲
1 Introduction to tumours of the digestive system
General introduction
Classification of neuroendocrine neoplasms of the digestive system
TNM staging of well-differentiated neuroendocrine tumours
2 Tumours of the oesophagus
WHO classification of tumours of the oesophagus
TNM staging of tumours of the oesophagus
Introduction
Benign epithelial tumours and precursors
Squamous papilloma
Barrett （related） dysplasia
Squamous dysplasia
Malignant epithelial tumours
Adenocarcinoma of the oesophagus and oesophagogastric junction NOS
Adenoid cystic carcinoma
Adenosquamous and mucoepidermoid carcinomas Squamous cell carcinoma NOS
Undifferentiated carcinoma
Neuroendocrine neoplasms
3 Tumours of the stomach
WHO classification of tumours of the stomach
TNM staging of carcinomas of the stomach
Introduction
Gastritis and metaplasia precursors of gastric neoplasms
Benign epithelial tumours and precursors
Fundic gland polyps
Hyperplastic polyps
Dysplasia
Intestinal-type adenoma Foveolar-type adenoma
Pyloric gland adenoma
Oxyntic gland adenoma
Malignant epithelial tumours
Adenocarcinoma
Squamous cell carcinoma
Adenosquamous carcinoma
1消化系统肿瘤介绍
总论
消化系统神经内分泌肿瘤分类
分化良好神经内分泌肿瘤TNM分期
2 食管肿瘤
食管肿瘤WHO分类
食管肿瘤TNM分期
概述
良性上皮性肿瘤和癌前病变
鳞状上皮乳头状瘤
巴雷特（相关性）异型增生
鳞状上皮异型增生
恶性上皮性肿瘤
食管与食管胃交界处腺癌 非特殊类型
腺样囊性癌
腺鳞癌和黏液表皮癌
鳞状细胞癌 非特殊类型
未分化癌
神经内分泌肿瘤
3 胃肿瘤
胃肿瘤WHO分类
胃癌TNM分期
概述
胃炎和胃肿瘤癌前化生性病变
良性上皮性肿瘤和癌前病变
胃底腺息肉
增生性息肉
非典型增生
肠型腺瘤
平坦型腺瘤
胃主腺腺瘤
泌酸性腺瘤
恶性上性肿瘤
腺癌
鳞状细胞癌
腺鳞癌
Undifferentiated carcinoma
Gastroblastoma
Neuroendocrine neoplasms
4 Tumours of the small intestine and ampulla
WHO classification of tumours of the small intestine and ampulla
TNM staging of carcinomas of the small intestine
TNM staging of carcinomas of the ampulla of Vater
Introduction
Benign epithelial tumours and precursors
Non-ampullary adenoma
Ampullary adenoma
Malignant epithelial tumours
Non-ampullary adenocarcinoma
Ampullary adenocarcinoma
Neuroendocrine neoplasms
5 Tumours of the appendix
WHO classification of tumours of the appendix
TNM staging of adenocarcinomas of the appendix
Introduction
Epithelial tumours
Serrated lesions and polyps
Mucinous neoplasm
Adenocarcinoma
Goblet cell adenocarcinoma
Neuroendocrine neoplasms
6 Tumours of the colon and rectum
WHO classification of tumours of the colon and rectum
TNM staging of carcinomas of the colon and rectum
lntroduction
Benign epithelial tumours and precursors
Serrated lesions and polyps
Conventional adenoma
Inflammatory bowel disease-associated dysplasia
Malignant epithelial tumours
Adenocarcinoma
Neuroendocrine neoplasms
未分化癌
胃母细胞瘤
神经内分泌肿瘤
4小肠和壶腹部肿瘤
小肠和壶腹部肿瘤的WHO肿瘤分类
小肠癌的TNM分期
瓦特壶腹部癌的TNM分期
介绍
良性和癌前病变
非壶腹部腺瘤
壶腹部腺瘤
恶性肿瘤
非壶腹部腺癌
壶腹部腺癌
神经内分泌肿瘤
5 阑尾肿瘤
阑尾的WHO肿瘤分类
阑尾腺癌的TNM分期
介绍
上皮性肿瘤
锯齿状腺瘤和息肉
黏液性肿瘤
腺癌
杯状细胞腺癌
神经内分泌肿瘤
6 结直肠肿瘤
结直肠肿瘤的WHO肿瘤分类
结直肠肿瘤的的TNM分期
介绍
良性上皮性肿瘤和癌前病变
锯齿状腺瘤与息肉
传统型腺瘤
炎症性肠病相关性异型增生
恶性上皮性肿瘤
腺癌
神经内分泌肿瘤
7 Tumours of the anal canal
WHO classification of tumours of the anal canal
TNM staging of tumours of the anal canal and perianal skin
Introduction
Benign epithelial tumours and precursors
Inflammatory cloacogenic polyp
Condyloma
Squamous dysplasia（intraepithelial neoplasia)
Malignant epithelial tumours
Squamous cell carcinoma
Adenocarcinoma
Neuroendocrine neoplasms
8 Tumours of the liver and intrahepatic bile ducts
WHO classification of tumours of the liver and intrahepatic bile ducts
TNM staging of tumours of the liver
TNM staging of tumours of the intrahepatic bile ducts
Introduction
Benign hepatocellular tumours
Focal nodular hyperplasia of the liver
Hepatocellular adenoma
Malignant hepatocellular tumours and precursors
Hepatocellular carcinoma
Hepatoblastoma
Benign biliary tumours and precursors
Bile duct adenoma
Biliary adenofibroma
Mucinous cystic neoplasm of the liver and biliary system
Billiary intraepithelial neoplasia (see Ch 9)
lntraductal papillary neoplasm of the bile ducts(see Ch. 9)
Malignant biliary tumours
lntrahepatic cholangiocarcinoma
Combined hepatocellular-cholangiocarcinoma and
undifterentiated primary liver carcinoma
Hepatic neuroendocrine neoplasms
7 肛管肿瘤
肛管肿瘤的WHO分类
肛管和肛周皮肤肿瘤的TNM分期
简介
良性上皮性肿瘤和癌前病变
炎性泄殖腔息肉
尖锐湿疣
鳞状上皮异型增生（上皮内瘤变）
恶性上皮性肿瘤
鳞状细胞癌
腺癌
神经内分泌肿瘤
8 肝和肝内胆管肿瘤
肝脏和肝内胆管肿瘤的WHO分类
肝脏肿瘤TNM分期
肝内胆管肿瘤TNM分期
良性肝细胞性肿瘤
肝局限性结节性增生
肝细胞性腺瘤
恶性肝细胞性肿瘤和癌前病变
肝细胞肝癌
肝母细胞瘤
良性胆道肿瘤和癌前病变
胆管腺瘤
胆管腺纤维瘤
肝脏和胆道系统黏液囊性肿瘤
胆管上皮内瘤变（见第九章）
胆管导管内乳头状瘤（见第九章）
恶性胆道肿瘤
肝内胆管癌
合并肝细胞胆管癌和未分化原发性肝癌
肝内神经内分泌肿瘤
9 Tumours of the gallbladder and extrahepatic bile ducts
WHO classification of tumours of the gallbladder and extrahepatic bile ducts
TNM staging of tumours of the gallbladder
TNM staging of tumours of the perihilar bile ducts
TNM staging of tumours of the distal extrahepatic bile duct
Introduction
Benign epithelial tumours and precursors
Pyloric gland adenoma of the gallbladder
Biliary intraepithelial neoplasia
Intracholecystic papillary neoplasm
Intraductal papillary neoplasm of the bile ducts
Mucinous cystic neoplasm of the liver and biliary system
(see Ch. 8)
Malignant epithelial tumours
Carcinoma of the gallbladder
Carcinoma of the extrahepatic bile ducts
Neuroendocrine neoplasms of the gallbladder and bile ducts
10 Tumours of the pancreas
WHO classification of tumours of the pancreas
TNM staging of carcinomas of the pancreas
Introduction
Benign epithelial tumours and precursors
Acinar cystic transformation
Serous neoplasms
Intraepithelial neoplasia
Intraductal papillary mucinous neoplasm
Intraductal oncocytic papillary neoplasm
Intraductal tubulopapillary neoplasm
Mucinous cystic neoplasm
Malignant epithelial tumours
Ductal adenocarcinoma
Acinar cell carcinoma
Pancreatoblastoma
Solid pseudopapillary neoplasm
9 胆囊和肝外胆管肿瘤
胆囊和肝外胆管肿瘤WHO分类
胆囊肿瘤TNM分期
门周胆管肿瘤TNM分期
肝外胆管肿瘤TNM分期
简介
良性上皮肿瘤和癌前病变
胆囊幽门腺瘤
胆囊上皮内瘤变
胆囊内乳头状肿瘤
胆管导管内乳头状肿瘤
肝和胆道系统黏液囊性肿瘤
（见第八章）
恶性上皮性肿瘤
胆囊癌
肝外胆管癌
胆囊和胆管神经内分泌肿瘤
10 胰腺肿瘤
胰腺肿瘤WHO分类
胰腺癌的TNM分期
介绍
良性上皮性肿瘤和癌前病变
腺泡囊性病
浆液性肿瘤
上皮内瘤变
导管内乳头黏液性肿瘤
导管内嗜酸性乳头状瘤
导管内管状乳头状肿瘤
黏液囊性肿瘤
恶性上皮性肿瘤
导管腺癌
腺泡细胞癌
胰母细胞瘤
实性假乳头状肿瘤
Neuroendocrine neoplasms
Introduction
Non-functioning pancreatic neuroendocrine tumours
Functioning neuroendocrine tumours
Insulinoma
Gastrinoma
VIPoma
Glucagonoma
Somatostatinoma
ACTH-producing neuroendocrine tumour
Serotonin-producing. neuroendocrine tumour
Neuroendocrine carcinoma
MiNENs
11 Haematolymphoid tumours of the digestive system
WHO classification of haematolymphoid tumours
of the digestive system
Introduction
Site-specific haematolymphoid tumours
Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)
Duodenal-type follicular lymphoma
Enteropathy-associated T-cell lymphoma
Monomorphic epitheliotropic I
Intestinal T-cell lymphoma
Intestinal T-cell lymphoma NOS
Indolent T-cell lvmphoproliferative disorder of the Gl tract
Hepatosplenic T-cell lymphoma
EBV+ inflammatory follicular dendritic cell sarcoma
Haematolymphoid tumours occurring with some frequency in the digestive system
Diffuse large B-cell lymphoma
Follicular lymphoma
Mantle cell lymphoma
Burkitt lymphoma
Plasmablastic lymphoma
Posttransplant lymphoproliferative disorders
Extranodal NK/T-cell lymphoma
Systemic mastocytosis
Langerhans cell histiocytosis
神经内分泌肿瘤
简介
非功能性胰腺神经内分泌肿瘤
功能性神经内分泌肿瘤
胰岛瘤
胃泌素瘤
胰肽瘤
胰高血糖瘤
生长抑制素瘤
产生ACTH神经内分泌肿瘤
产生羟色胺神经内分泌肿瘤
神经内分泌癌
混合型神经内分泌非神经内分泌肿瘤
11 消化系统淋巴造血肿瘤
世界卫生组织对消化系统血液淋巴瘤的分类
消化系统血液肿瘤的分类
简介
部位特异性淋巴造血肿瘤
黏膜相关淋巴组织的结节外边缘区淋巴瘤（MALT淋巴瘤）
十二指肠型滤泡淋巴瘤
肠病相关的T细胞淋巴瘤
单形上皮性肠道T细胞淋巴瘤
肠道T细胞淋巴瘤NOS
慢性T细胞淋巴增生性胃肠道疾病
肝脾T细胞淋巴瘤
EBV+炎症性滤泡树突状细胞肉瘤
在消化系统中以一定频率发生的血液淋巴肿瘤
弥漫性大B细胞淋巴瘤
滤泡淋巴瘤
套细胞淋巴瘤
伯基特淋巴瘤
浆母细胞性淋巴瘤
移植后淋巴增生性疾病
节外NK/T细胞淋巴瘤
系统性肥大细胞增多症
朗格汉斯细胞组织细胞增生症
Follicular dendritic cell sarcoma
Histiocytic sarcoma
滤泡树突状细胞肉瘤
组织细胞性肉瘤
Foreword
The WHO Classification of Tumours, published as a series of books (also known as the WHO Blue Books), is an essential tool for standardizing diagnostic practice worldwide. The WHO classification also serves as a vehicle for the translation of cancer research into practice. The diagnostic criteria and standards these books contain are underpinned by evidence evaluated and debated b experts in the field. About 200 authors and editors participate in the production of each book, and they give their time freely to this task. I am very grateful for their help; it is a remarkable team effort.
This first volume of the fifth edition of the WHO Blue Books incorporates several important changes to the series as a whole. For example, this is the first WHO Blue Book to be led by an editorial board. The WHO Classification of Tumours Editorial Board is composed of standing members nominated by pathology organizations and expert members selected on the basis of informed bibliometric analysis. The diagnostic process is increasingly multidisciplinary, and we are delighted that several radiology and clinical experts have already joined us to address specific needs. The editorial board also includes a patient representative.
The most conspicuous change to the format of the books in the fifth edition is that tumour types common to multiple svstems are dealt with together - so there are separate chapters on haematolymphoid tumours and mesenchymal tumours. There is also a chapter on genetic tumour syndromes. Genetic disorders are of increasing importance to diagnosis in individual patients, and the study of these disorders has undoubtedly informed our understanding of tumour biology and behaviour over the past 10 years. The inclusion of a chapter dedicated to genetic
前言
以丛书形式出版的《世界卫生组织肿瘤分类》（也称为《世界卫生组织蓝皮书》）是在全世界范围内实现诊断实践标准化的一个重要工具。世卫组织的分类也是将癌症研究转化为实践的工具。这些书中包含的诊断标准和规范是由该领域的专家评估和辩论的证据所支撑的。大约有200名作者和编辑参与了每本书的制作，他们为这项工作无偿奉献了自己的时间。我非常感谢他们的帮助；这是一个了不起的团队努力。
世卫组织蓝皮书第五版的第一卷包含了整个系列的几个重要变化。例如，这是第一本由编辑委员会领导的世卫组织蓝皮书。世卫组织肿瘤分类编辑委员会由病理组织提名的常设成员和在知情的文献计量分析基础上选出的专家成员组成。诊断过程越来越具有多学科性，我们很高兴一些放射学和临床专家已经加入我们，以解决具体的需求。编辑委员会还包括一名患者代表。
第五版中书籍格式最明显的变化是将多个系统中常见的肿瘤类型放在一起处理--因此有关于血液淋巴瘤和间质瘤的单独章节。此外，还有一章是关于遗传性肿瘤综合症的。遗传性疾病对个别病人的诊断越来越重要，对这些疾病的研究无疑为我们在过去10年里对肿瘤生物学和行为的理解提供了依据。列入专门讨论遗传性肿瘤综合征的章节反映了这种重要性。
tumour syndromes reflects this importance.
We have attempted to take a more systematic approach to the multifaceted nature of tumour classification: each tumour type is described on the basis of its localization, clinical features, epidemiology, etiology, pathogenesis, histopathology, diagnostic molecular pathology, staging, and prognosis and prediction. Where appropriate we have also included information on macroscopic appearance and cytology, as well as essential and desirable diagnostic criteria. This standardized, modular approach is in part to ready the books to be accessible online, but it also enables us to call attention to areas in which there is little information, and where serious gaps in our knowledge remain to be addressed
The organization of the WHO Blue Books content now follows the normal progression from benign to malignant - a break with the fourth edition, but one we hope will be welcome.
The volumes are still organized on the basis of anatomical site (digestive system, breast, soft tissue and bone, etc.), and each tumour type is listed within a taxonomic classification that follows the format below. which helps to structure the books in a systematic manner:
• Site; e.g. stomach
• Category; e.g. epithelial tumours
• Family (class); e.g. adenomas and other premalignant neoplastic lesions
• Type; e.g. adenoma
• Subtype; e.g. foveolar-type adenoma
The issue of whether a given tumour type represents a distinct entity rather than a subtype continues to exercise pathologists, and it is the topic of many publications in the scientific literature.
我们试图采取一种更加系统的方法来处理肿瘤分类的多面性：根据其定位、临床特征、流行病学、病因学、发病机制、组织病理学、诊断性分子病理学、分期以及预后和预测来描述每种肿瘤类型。在适当的地方，我们还包括宏观外观和细胞学方面的信息，以及基本和理想的诊断标准。这种标准化、模块化的方法，部分是为了使书籍能够在网上查阅，但它也使我们能够提请注意那些信息很少的领域，以及我们的知识中存在的严重差距有待解决的地方。
世卫组织蓝皮书的内容组织现在遵循从良性到恶性的正常进展--这是与第四版的一个突破，但我们希望这将是受欢迎的。
各卷仍按解剖部位（消化系统、乳腺、软组织和骨骼等）编排，每一种肿瘤类型都按照以下格式列在分类法中，这有助于以系统的方式组织书籍。
- 部位；例如：胃
- 类别；如：上皮性肿瘤
- 科（类）；如腺瘤和其他恶性肿瘤前期病变
- 类型；如：腺瘤
- 亚型；如小凹型腺瘤
一个特定的肿瘤类型是否代表一个独特的实体而不是一个亚型，这个问题继续困扰着病理学家，这也是科学文献中许多出版物的主题。
We continue to deal with this issue on a case-by-case basis, but we believe there are inherent rules that can be applied. For example, tumours in which multiple histological patterns contain shared truncal mutations are clearly of the same type, despite the differences in their appearance. Equally, genetic heterogeneity within the same tumour type may have implications for treatment. A small shift in terminology as of this new edition is that the term "variant" in reference to a specific kind of tumour has been wholly superseded by "subtype", in an effort to more clearly differentiate this meaning from that of "variant" in reference to a genetic alteration.
The WHO Blue Books are much appreciated by pathologists and of increasing importance to cancer researchers. The new editorial board and I certainly hope that the series will continue to meet the need for standards in diagnosis and to facilitate the translation of diagnostic research into practice worldwide. It is particularly important that cancers continue to be classified and diagnosed according to the same standards internationally so that patients can benefit from multicentre clinical trials, as well as from the results of local trials conducted on different continents.
Dr lan A. Cree
Head, WHO Classification of Tumours Group International Agency for Research on Cancer
June 2019
我们继续在个案的基础上处理这个问题，但我们相信有一些固有的规则可以应用。例如，多个组织学模式包含共同的干细胞突变的肿瘤显然属于同一类型，尽管它们在外观上存在差异。同样，同一肿瘤类型中的遗传异质性也可能对治疗产生影响。新版蓝皮书在术语上的一个小变化是，提到一种特定的肿瘤时，"变异 "一词已完全被 "亚型 "所取代，目的是为了更明确地区分这一含义与提到基因改变时的 "变异 "含义。
世卫组织蓝皮书深受病理学家的赞赏，对癌症研究人员来说也越来越重要。新的编委会和我当然希望这套丛书能继续满足诊断标准的需要，并促进诊断研究在全世界范围内的实践转化。尤其重要的是，在国际上继续按照相同的标准对癌症进行分类和诊断，以便患者能够从多中心的临床试验以及在不同大洲进行的本地试验结果中受益。
lan A. Cree博士
世界卫生组织肿瘤分类组组长 国际癌症研究机构
2019年6月