Fischer Hans-Peter, Zhou Hui
Department of Pathology, University of Bonn, Sigmund-Freud-Str. 25, D-53127, Bonn, Germany.
J Hepatobiliary Pancreat Surg. 2004;11(5):301-9. doi: 10.1007/s00534-004-0898-3.
Most adenomas and carcinomas of the small intestine and extrahepatic bile ducts arise in the region of the papilla of Vater. In familial adenomatous polyposis (FAP) it is the main location for carcinomas after proctocolectomy. In many cases symptoms due to stenosis lead to diagnosis at an early tumor stage. In about 80%, curative intended resection is possible. Operability is the most relevant prognostic factor. Most ampullary carcinomas resp. carcinomas of the papilla of Vater develop from adenomatous or flat dysplastic precursor lesions. They can be sited in the ampulloduodenal part of the papilla of Vater, which is lined by intestinal mucosa. They also can develop in deeper parts of the ampulla, which are lined by pancreaticobiliary duct mucosa. Intestinal-type adenocarcinoma and pancreaticobiliary-type adenocarcinoma represent the main histological types of ampullary carcinoma. Furthermore, there exist unusual types and undifferentiated carcinomas. Many carcinomas of intestinal type express the immunohistochemical marker profile of intestinal mucosa (keratin 7-, keratin 20+, MUC2+). Carcinomas of pancreaticobiliary type usually show the immunohistochemical profile of pancreaticobiliary duct mucosa (keratin 7+, keratin 20-, MUC2-). Even poorly differentiated carcinomas, as well as unusual histological types, may conserve the marker profile of the mucosa they developed from. These findings underline the concept of histogenetically different carcinomas of the papilla of Vater which develop either from intestinal- or from pancreaticobiliary-type mucosa of the papilla of Vater. Molecular alterations in ampullary carcinomas are similar to those of colorectal as well as pancreatic carcinomas, although they appear at different frequencies. In future studies, molecular alterations in ampullary carcinomas should be correlated closely with the different histologic tumor types. Consequently, the histologic classification should reflect the histogenesis of ampullary tumors from the two different types of papillary mucosa.
大多数小肠和肝外胆管的腺瘤及癌起源于 Vater 壶腹区域。在家族性腺瘤性息肉病（FAP）中，它是直肠结肠切除术后癌的主要发生部位。在许多情况下，由于狭窄导致的症状会在肿瘤早期阶段引发诊断。约 80%的病例可行根治性切除。可切除性是最相关的预后因素。大多数壶腹癌或 Vater 壶腹癌由腺瘤性或扁平发育异常的前驱病变发展而来。它们可位于 Vater 壶腹的壶腹十二指肠部分，该部分由肠黏膜覆盖。它们也可在壶腹的更深部位发生，此处由胰胆管黏膜覆盖。肠型腺癌和胰胆管型腺癌是壶腹癌的主要组织学类型。此外，还存在不常见类型和未分化癌。许多肠型癌表达肠黏膜的免疫组化标志物谱（角蛋白 7阴性、角蛋白 20阳性、MUC-）。胰胆管型癌通常显示胰胆管黏膜的免疫组化谱（角蛋白 7阳性、角蛋白 20阴性、MUC-）。即使是低分化癌以及不常见的组织学类型，也可能保留其起源黏膜的标志物谱。这些发现强调了 Vater 壶腹组织发生学上不同的癌的概念，这些癌要么起源于 Vater 壶腹的肠型黏膜，要么起源于胰胆管型黏膜。壶腹癌的分子改变与结直肠癌以及胰腺癌相似，尽管它们出现的频率不同。在未来的研究中，壶腹癌的分子改变应与不同的组织学肿瘤类型密切相关。因此，组织学分类应反映壶腹肿瘤从两种不同类型乳头黏膜的组织发生。
