2019WHO消化系統腫瘤分類 第五版 大綱、前言翻譯
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僅代表個人閱讀軌跡，英文內容不全面，翻譯內容僅供參考。
大綱
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消化系統腫瘤介紹
總論
消化系統神經內分泌腫瘤分類
分化良好神經內分泌腫瘤TNM分期
2 食管腫瘤
食管腫瘤WHO分類
食管腫瘤TNM分期
概述
良性上皮性腫瘤和癌前病變
鱗狀上皮乳頭狀瘤
巴雷特（相關性）異型增生
鱗狀上皮異型增生
惡性上皮性腫瘤
食管與食管胃交界處腺癌 非特殊類型
腺樣囊性癌
腺鱗癌和黏液表皮癌
鱗狀細胞癌 非特殊類型
未分化癌
神經內分泌腫瘤
3 胃腫瘤
胃腫瘤WHO分類
胃癌TNM分期
概述
胃炎和胃腫瘤癌前化生性病變
良性上皮性腫瘤和癌前病變
胃底腺息肉
增生性息肉
非典型增生
腸型腺瘤
平坦型腺瘤
胃主腺腺瘤
泌酸性腺瘤
惡性上性腫瘤
腺癌
鱗狀細胞癌
腺鱗癌
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
胃母細胞瘤
神經內分泌腫瘤
4小腸和壺腹部腫瘤
小腸和壺腹部腫瘤的WHO腫瘤分類
小腸癌的TNM分期
瓦特壺腹部癌的TNM分期
介紹
良性和癌前病變
非壺腹部腺瘤
壺腹部腺瘤
惡性腫瘤
非壺腹部腺癌
壺腹部腺癌
神經內分泌腫瘤
5 闌尾腫瘤
闌尾的WHO腫瘤分類
闌尾腺癌的TNM分期
介紹
上皮性腫瘤
鋸齒狀腺瘤和息肉
黏液性腫瘤
腺癌
杯狀細胞腺癌
神經內分泌腫瘤
6 結直腸腫瘤
結直腸腫瘤的WHO腫瘤分類
結直腸腫瘤的的TNM分期
介紹
良性上皮性腫瘤和癌前病變
鋸齒狀腺瘤與息肉
傳統型腺瘤
炎癥性腸病相關性異型增生
惡性上皮性腫瘤
腺癌
神經內分泌腫瘤
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 肛管腫瘤
肛管腫瘤的WHO分類
肛管和肛周皮膚腫瘤的TNM分期
簡介
良性上皮性腫瘤和癌前病變
炎性泄殖腔息肉
尖銳濕疣
鱗狀上皮異型增生（上皮內瘤變）
惡性上皮性腫瘤
鱗狀細胞癌
腺癌
神經內分泌腫瘤
8 肝和肝內膽管腫瘤
肝臟和肝內膽管腫瘤的WHO分類
肝臟腫瘤TNM分期
肝內膽管腫瘤TNM分期
良性肝細胞性腫瘤
肝局限性結節性增生
肝細胞性腺瘤
惡性肝細胞性腫瘤和癌前病變
肝細胞肝癌
肝母細胞瘤
良性膽道腫瘤和癌前病變
膽管腺瘤
膽管腺纖維瘤
肝臟和膽道系統黏液囊性腫瘤
膽管上皮內瘤變（見第九章）
膽管導管內乳頭狀瘤（見第九章）
惡性膽道腫瘤
肝內膽管癌
合并肝細胞膽管癌和未分化原發性肝癌
肝內神經內分泌腫瘤
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 膽囊和肝外膽管腫瘤
膽囊和肝外膽管腫瘤WHO分類
膽囊腫瘤TNM分期
門周膽管腫瘤TNM分期
肝外膽管腫瘤TNM分期
簡介
良性上皮腫瘤和癌前病變
膽囊幽門腺瘤
膽囊上皮內瘤變
膽囊內乳頭狀腫瘤
膽管導管內乳頭狀腫瘤
肝和膽道系統黏液囊性腫瘤
（見第八章）
惡性上皮性腫瘤
膽囊癌
肝外膽管癌
膽囊和膽管神經內分泌腫瘤
10 胰腺腫瘤
胰腺腫瘤WHO分類
胰腺癌的TNM分期
介紹
良性上皮性腫瘤和癌前病變
腺泡囊性病
漿液性腫瘤
上皮內瘤變
導管內乳頭黏液性腫瘤
導管內嗜酸性乳頭狀瘤
導管內管狀乳頭狀腫瘤
黏液囊性腫瘤
惡性上皮性腫瘤
導管腺癌
腺泡細胞癌
胰母細胞瘤
實性假乳頭狀腫瘤
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
神經內分泌腫瘤
簡介
非功能性胰腺神經內分泌腫瘤
功能性神經內分泌腫瘤
胰島瘤
胃泌素瘤
胰肽瘤
胰高血糖瘤
生長抑制素瘤
產生ACTH神經內分泌腫瘤
產生羥色胺神經內分泌腫瘤
神經內分泌癌
混合型神經內分泌非神經內分泌腫瘤
11 消化系統淋巴造血腫瘤
世界衛生組織對消化系統血液淋巴瘤的分類
消化系統血液腫瘤的分類
簡介
部位特異性淋巴造血腫瘤
黏膜相關淋巴組織的結節外邊緣區淋巴瘤（MALT淋巴瘤）
十二指腸型濾泡淋巴瘤
腸病相關的T細胞淋巴瘤
單形上皮性腸道T細胞淋巴瘤
腸道T細胞淋巴瘤NOS
慢性T細胞淋巴增生性胃腸道疾病
肝脾T細胞淋巴瘤
EBV+炎癥性濾泡樹突狀細胞肉瘤
在消化系統中以一定頻率發生的血液淋巴腫瘤
彌漫性大B細胞淋巴瘤
濾泡淋巴瘤
套細胞淋巴瘤
伯基特淋巴瘤
漿母細胞性淋巴瘤
移植后淋巴增生性疾病
節外NK/T細胞淋巴瘤
系統性肥大細胞增多癥
朗格漢斯細胞組織細胞增生癥
Follicular dendritic cell sarcoma
Histiocytic sarcoma
濾泡樹突狀細胞肉瘤
組織細胞性肉瘤
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
以叢書形式出版的《世界衛生組織腫瘤分類》（也稱為《世界衛生組織藍皮書》）是在全世界范圍內實現診斷實踐標準化的一個重要工具。世衛組織的分類也是將癌癥研究轉化為實踐的工具。這些書中包含的診斷標準和規范是由該領域的專家評估和辯論的證據所支撐的。大約有200名作者和編輯參與了每本書的制作，他們為這項工作無償奉獻了自己的時間。我非常感謝他們的幫助；這是一個了不起的團隊努力。
世衛組織藍皮書第五版的第一卷包含了整個系列的幾個重要變化。例如，這是第一本由編輯委員會領導的世衛組織藍皮書。世衛組織腫瘤分類編輯委員會由病理組織提名的常設成員和在知情的文獻計量分析基礎上選出的專家成員組成。診斷過程越來越具有多學科性，我們很高興一些放射學和臨床專家已經加入我們，以解決具體的需求。編輯委員會還包括一名患者代表。
第五版中書籍格式最明顯的變化是將多個系統中常見的腫瘤類型放在一起處理--因此有關于血液淋巴瘤和間質瘤的單獨章節。此外，還有一章是關于遺傳性腫瘤綜合癥的。遺傳性疾病對個別病人的診斷越來越重要，對這些疾病的研究無疑為我們在過去10年里對腫瘤生物學和行為的理解提供了依據。列入專門討論遺傳性腫瘤綜合征的章節反映了這種重要性。
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.
我們試圖采取一種更加系統的方法來處理腫瘤分類的多面性：根據其定位、臨床特征、流行病學、病因學、發病機制、組織病理學、診斷性分子病理學、分期以及預后和預測來描述每種腫瘤類型。在適當的地方，我們還包括宏觀外觀和細胞學方面的信息，以及基本和理想的診斷標準。這種標準化、模塊化的方法，部分是為了使書籍能夠在網上查閱，但它也使我們能夠提請注意那些信息很少的領域，以及我們的知識中存在的嚴重差距有待解決的地方。
世衛組織藍皮書的內容組織現在遵循從良性到惡性的正常進展--這是與第四版的一個突破，但我們希望這將是受歡迎的。
各卷仍按解剖部位（消化系統、乳腺、軟組織和骨骼等）編排，每一種腫瘤類型都按照以下格式列在分類法中，這有助于以系統的方式組織書籍。
- 部位；例如：胃
- 類別；如：上皮性腫瘤
- 科（類）；如腺瘤和其他惡性腫瘤前期病變
- 類型；如：腺瘤
- 亞型；如小凹型腺瘤
一個特定的腫瘤類型是否代表一個獨特的實體而不是一個亞型，這個問題繼續困擾著病理學家，這也是科學文獻中許多出版物的主題。
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
我們繼續在個案的基礎上處理這個問題，但我們相信有一些固有的規則可以應用。例如，多個組織學模式包含共同的干細胞突變的腫瘤顯然屬于同一類型，盡管它們在外觀上存在差異。同樣，同一腫瘤類型中的遺傳異質性也可能對治療產生影響。新版藍皮書在術語上的一個小變化是，提到一種特定的腫瘤時，"變異 "一詞已完全被 "亞型 "所取代，目的是為了更明確地區分這一含義與提到基因改變時的 "變異 "含義。
世衛組織藍皮書深受病理學家的贊賞，對癌癥研究人員來說也越來越重要。新的編委會和我當然希望這套叢書能繼續滿足診斷標準的需要，并促進診斷研究在全世界范圍內的實踐轉化。尤其重要的是，在國際上繼續按照相同的標準對癌癥進行分類和診斷，以便患者能夠從多中心的臨床試驗以及在不同大洲進行的本地試驗結果中受益。
lan A. Cree博士
世界衛生組織腫瘤分類組組長 國際癌癥研究機構
2019年6月