The fifth edition of WHO classification of digestive system tumors: update and progress on biliary system tumors

doi:10.16139/j.1007-9610.2023.02.08

Abstract

Abstract:

Overall, there was little change in biliary system tumors of the 5th edition of WHO classification of digestive system tumors after update. The three-tiered classification of biliary intraepithelial neoplasia changed to two-tiered system: high-grade and low-grade, in the section of benign tumors and precancerous lesions. And both intracholecystic and intraductal papillary tumors associated with invasive carcinoma were added. The classification of intrahepatic bile duct tumors introduced parts of bile duct adenoma and bile duct adenofibroma, which were mentioned previously in the differential diagnosis with bile duct carcinoma. Intrahepatic cholangiocarcinoma was divided into small bile duct subtype and large bile duct subtype in the 5th edition of WHO classification, and the names of cholangiocellular carcinoma and cholangiole cell carcinoma were no longer recommended to use. Extrahepatic cholangiocarcinoma was listed as a separate part for the first time, including squamous cell carcinoma, adenosquamous carcinoma and undifferentiated carcinoma in addition to common histological types. Some new contents of molecular pathology in the 5th edition of WHO classification were added, which provided the basis for precise classification and diagnosis of tumors in biliary system.

Key words: Biliary system tumor, Pathological diagnosis, Tumor classification

CLC Number:

R735.8

LUO Fangxiu, MA Qianchen, YUAN Fei. The fifth edition of WHO classification of digestive system tumors: update and progress on biliary system tumors[J]. Journal of Surgery Concepts & Practice, 2023, 28(02): 124-131.

Figures/Tables 5

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分类	ICD-0 编码	分类	ICD-0编码
良性上皮性肿瘤及癌前病变		恶性上皮性肿瘤
胆囊幽门腺腺瘤	8140/0	胆囊癌
胆管上皮内瘤变，低级别	8148/0	腺癌，NOS	8140/3
胆管上皮内瘤变，高级别	8148/2	肠型腺癌	8144/3
胆囊内乳头状肿瘤伴低级别上皮内瘤变	8503/0	透明细胞癌，NOS	8310/3
胆囊内乳头状肿瘤伴低级别上皮内瘤变	8503/0	黏液腺癌	8480/3
胆囊内乳头状肿瘤伴高级别上皮内瘤变	8503/2	低黏附性癌伴或不伴印戒细胞	8490/3
胆管导管内乳头状肿瘤伴低级别上皮内瘤变	8503/0	胆囊内乳头状肿瘤伴相关浸润性癌	8503/3
胆管导管内乳头状肿瘤伴高级别上皮内瘤变	8503/2	黏液性囊性肿瘤伴相关浸润性癌	8470/3
		鳞状细胞癌，NOS	8070/3
		未分化癌，NOS	8020/3
		肝外胆管癌
		胆管癌	8160/3
		胆管导管内乳头状肿瘤伴相关浸润性癌	8503/3
		鳞状细胞癌，NOS	8070/3
		腺鳞癌	8560/3
		未分化癌，NOS	8020/3
		神经内分泌瘤，NOS	8240/3
		神经内分泌瘤，G1	8240/3
		神经内分泌瘤，G2	8249/3
		神经内分泌瘤，G3	8249/3
		神经内分泌癌，NOS	8246/3
		大细胞神经内分泌癌	8013/3
		小细胞神经内分泌癌	8041/3
		混合性神经内分泌-非神经内分泌肿瘤（MiNEN）	8154/3

特征	1型	2型
好发部位	肝内胆管	肝外胆管
胆管大体特征	囊性或圆柱状扩张	圆柱形或纺锤状扩张
黏液分泌	常见	罕见
组织学特征
被覆上皮	上皮规则、单一，乳头形态>管状形态	上皮不规则、复杂，以乳头状形态为主，呈筛状或实性生长模式
纤维轴心	纤细的纤维血管轴心	纤细血管，灶性纤维性间质
组织学亚型	胃型、肠型	肠型、胰胆管型
分级	多为高级别，伴局灶低级别区域	始终是高级别，时有灶性低级别区域
间质浸润	不常见（<50%），多为微小浸润，偶尔呈结节状	常见（>80%），呈轻、中程度浸润
与IPMN相似程度	相似	形态多样
进展可能性	较低	较高
预后	较好	比1型差

分类	ICD-0 编码	分类	ICD-0 编码
良性胆管肿瘤及癌前病变		胆管恶性上皮性肿瘤
胆管腺瘤	8160/0	肝内胆管癌	8160/3
胆管腺纤维瘤	9013/0	大胆管型胆管癌
胆管上皮内瘤变，低级别	8148/0	小胆管型胆管癌
胆管上皮内瘤变，高级别	8148/2	胆管导管内乳头状肿瘤伴相关浸润性癌	8503/3
胆管导管内乳头状肿瘤伴低级别上皮内瘤变	8503/0	黏液性囊性肿瘤伴相关浸润性癌	8470/3
胆管导管内乳头状肿瘤伴高级别上皮内瘤变	8503/2	未分化癌，NOS	8020/3
胆管导管内乳头状肿瘤伴高级别上皮内瘤变	8503/2	混合性肝细胞-胆管癌	8180/3
黏液性囊性肿瘤伴低级别上皮内瘤变	8470/0	神经内分泌瘤，NOS	8240/3
黏液性囊性肿瘤伴低级别上皮内瘤变	8470/0	神经内分泌瘤，G1	8240/3
黏液性囊性肿瘤伴高级别上皮内瘤变	8470/2	神经内分泌瘤，G2	8249/3
黏液性囊性肿瘤伴高级别上皮内瘤变	8470/2	神经内分泌瘤，G3	8249/3
		神经内分泌癌，NOS	8246/3
		大细胞神经内分泌癌	8013/3
		小细胞神经内分泌癌	8041/3
		混合性神经内分泌-非神经内分泌肿瘤（MiNEN）	8154/3

特征	iCCA		肝外胆管癌
特征	小胆管型	大胆管型	肝外胆管癌
细胞起源	肝内小胆管；可能与肝祖细胞有关	肝内大胆管或胆管周腺体	肝外胆管或胆管周腺体
形态学	被覆立方上皮的小导管状；罕见黏液分泌	分泌黏液的柱状细胞形成大导管状或乳头状	与大胆管型iCCA类似
免疫组织化学表型	共同表达CK7、CK19、EMA（MUC1）
免疫组织化学表型	表达C反应蛋白、N钙黏着素、CD56	表达S100、MUC5AC、MUC6、TFF1	与大胆管型iCCA类似
侵袭性	可形成卫星结节神经、脉管侵犯少见淋巴结转移少见	较少形成卫星结节神经、脉管侵犯多见肝内浸润性生长	与大胆管型iCCA类似
分子特征	IDH1/2、BRCA1/2、BAP1突变，FGFR2融合	KRAS、TP53突变	与大胆管型iCCA类似，KRAS突变频率更高