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Pathogenese und Histopathologie von Adenomen und Karzinomen der Papilla Vateri

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Zusammenfassung

Die Vater-Papille ist im Dünndarm und im Verlauf des Ductus choledochus der häufigste Entstehungsort von Adenomen und Karzinomen. Bei familiärer adenomatöser Polypose (FAP) ist sie Hauptsitz für Karzinome nach Proktokolektomie. Aufgrund früher stenosebedingter Symptome sind ca. 80% der Tumoren mit kurativer Intention chirurgisch angehbar. Die Operabilität ist der wichtigste Prognosefaktor; eine frühe Diagnose ist somit verlaufsentscheidend. Entzündung, regeneratorische Veränderungen und Fibrose nach endoskopischen Eingriffen, begleitende Hyperplasie, adenomatöse Vorläufer und tiefer Sitz mancher Papillenkarzinome erschweren die Karzinomdiagnose am Biopsiematerial. Als histologische Haupttypen lassen sich das intestinal und das pankreatobiliär differenzierte Papillenkarzinom unterscheiden. Hinzu kommen nicht weiter differenzierbare Karzinome und Sonderformen. Im eigenen Kollektiv 45 operierter Papillenkarzinome wiesen 6 der 10 Tumoren des intestinalen Typs und 4 Tumoren mit Sonderformen das Markerprofil intestinaler Schleimhaut auf (Keratin 7−, Keratin 20+, MUC2+). 17 von 21 Tumoren des pankreatobiliären Typs, alle 4 undifferenzierten Karzinome und 3 papilläre Karzinome zeigten das Markerprofil pankreatobiliärer Gangschleimhaut (Keratin 7+, Keratin 20−, MUC2−). Bei 3 mit diesen Marker nicht reagierenden Karzinome zeigten nichtinvasive Vorläuferläsionen eine der beiden Markerkombinationen. Diese Befunde unterstreichen das Konzept histogenetisch unterschiedlicher Papillenkarzinome, die vom intestinal oder vom pankreatobiliär differenzierten Schleimhautanteil der Vater-Papille ausgehen. Die Molekularpathogenese des Papillenkarzinoms ähnelt einerseits der des kolorektalen Karzinoms, andererseits des duktalen Pankreaskarzinoms, doch bestehen Unterschiede in der Häufigkeit der molekularen Alterationen. Zukünftige molekularpathologische Untersuchungen des Papillenkarzinoms sollten auf einer histologischen Klassifikation gründen, die die Histogenese des Papillenkarzinoms aus den 2 verschiedenen Schleimhauttypen der Papilla Vateri konsequent berücksichtigt.

Abstract

Most adenomas and carcinomas of small intestine and extrahepatic bile ducts arise in the region of Vater's papilla. In FAP it is the main location for carcinomas after proctocolectomy.

In many cases symptoms due to stenosis lead to diagnosis in an early tumor stage. In about 80% curative intended resection is possible. Operability is the most relevant prognostic factor. Inflammatory changes, fibrosis, regeneratory changes after endoscopic manipulation, hyperplasia, preneoplastic lesions close to carcinoma, deeply sited carcinomas under protruded, non-neoplastic duodenal mucosa make the diagnosis difficult on biopsy material. Histologically, intestinal type adenocarcinoma, pancreatobiliary type adenocarcinoma, undifferentiated carcinomas and unusual types can be differentiated. In our own series comprising 45 resected ampullary carcinomas 6 from 10 intestinal type adenocarcinomas, and 4 carcinomas of unusual types expressed the immunohistochemical marker profile of intestinal mucosa (keratin 7−, keratin 20+, MUC2+). 17 from 21 pancreatobiliary type adenocarcinomas, 4 undifferentiated carcinomas, as well as 3 papillary carcinomas showed the immunohistochemical profile of pancreaticobiliary duct mucosa (keratin 7+, keratin 20−, MUC2−). 3 invasive carcinomas which were negative for these markers, showed one of these characteristic marker-combinations in non-invasive adenomatous parts. These findings support the concept of histogenetically different ampullary carcinomas which are developing from the intestinal or from the pancreaticobiliary type mucosa of Vater's papilla. Molecular alterations in ampullary carcinomas are similar to those of colorectal as well as pancreatic carcinomas, although they appear in different frequencies. In future studies molecular alterations in ampullary carcinomas should be correlated closely with the different histologic tumor types. The histologic classification should reflect consequently the histogenesis of ampullary tumors from the two different types of papillary mucosa.

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Literatur

  1. Achille A, Scupoli MT, Magalini A, Zamboni G, Romanelli MG, Orlandini S, Biasi MO, Lemoine NR, Accola RS, Scarpa A (1996) APC gene mutations and allelic losses in sporadic ampullary tumours: evidence of genetic difference from tumours associated with familial adenomatous polyposis. Int J Cancer 68:305–312

    Article  CAS  PubMed  Google Scholar 

  2. Achille A, Biasi MO, Zamboni G, Bogina G, Iacono C, Talamini G, Capella G, Scarpa A (1997) Cancers of the papilla of Vater: mutator phenotype is associated with good prognosis. Clin Cancer Res 3:1841–1847

    CAS  PubMed  Google Scholar 

  3. Achille A, Baron A, Zamboni G, Di Pace C, Orlandini S, Scarpa A (1998) Chromosome 5 allelic losses are early events in tumours of the papilla of Vater and occur at sites similar to those of gastric cancer. Brit J Cancer 78:1653–1660

    CAS  PubMed  Google Scholar 

  4. Agoff SN, Crispin DA, Bronner MP, Dail DH, Hawes SE, Haggitt RC (2001) Neoplasms of the ampulla of Vater with concurrent pancreatic intraductal neoplasia: a histological and molecular study. Mod Pathol 14:139–146

    CAS  PubMed  Google Scholar 

  5. Albores-Saavedra J, Henson DE, Klimstra DS (2000) Tumors of the gallbladder, extrahepatic bile ducts, and ampulla of Vater. Atlas of tumor pathology, 3rd series, Fascicle 27. AFIP Warhi

  6. Albores-Saavedra J, Menck HR, Scoazec JC, Soehendra N, Wittekind C, Sriram N, Sripa B (2000) Tumours of the gallbladder and extrahepatic bile ducts. In: Hamilton SR, Aaltonen LA (eds) WHO classification of tumours. Pathology and genetics of the digestive system. IARC Press Lyon, pp 203–218

  7. Andiran F, Tanyel FC, Kale G, Akhan O, Akcoren Z, Hicsonmez A (1997) Obstructive jaundice resulting from adenocarcinoma of the Vater's ampulla. Rev Enferm Dig 85:391–393

    Google Scholar 

  8. Austin JC, Organ CH, Williams GR, Pitha JV (1988) Vaterian cancer in siblings. Ann Surg 207:644–661

    Google Scholar 

  9. Burke CA, Beck GJ, Church JM, van Stolk RU (1999) The natural history of untreated duodenal and ampullary adenomas in patients with familial adenomatous polyposis followed in an endoscopic surveillance program. Gastrointest Endosc 49:358–364

    CAS  PubMed  Google Scholar 

  10. Chung CH, Wilentz RE, Polak MM, Ramsoekh TB, Noorduyn LA, Gouma DJ, Huibregtse K, Offerhaus GJA, Siebos RJC (1996) Clinical significance of K-ras oncogene activation in ampullary neoplasms. Clin Pathol 49:460–464

    CAS  Google Scholar 

  11. Costi R, Caruana P, Sarli L, Violi V, Roncoroni L, Bordi C (2001) Ampullary adenocarcinoma in neurofibromatosis type 1. Case report and literature review. Mod Pathol 14:1169–1174

    CAS  PubMed  Google Scholar 

  12. Finklestein SD, Sayegh, R, Christensen S, Swalsky PA (1993) Genotypic classification of colorectal adenocarcinoma. Cancer 71:3827–3838

    PubMed  Google Scholar 

  13. Fischer HP, Zhou H (2000) Pathologie der Papilla Vateri. In: Doerr W, Seifert G, Uehlinger E (Hrsg) Spezielle Pathologische Anatomie: Pathologie der Leber und der Gallenwege, 2. Aufl. Springer, Berlin Heidelberg New York, S 1219–1257

    Google Scholar 

  14. Galle TS, Juel K, Bülow S (1999) Causes of death in familial adenomatous polyposis. Scand J Gastroenterol 34:808–812

    Article  CAS  PubMed  Google Scholar 

  15. Howe JR, Klimstra DS, Cordon-Cardo C, Paty PB, Park PY, Brennan MF (1997) K-ras mutation in adenomas and carcinomas of the ampulla of Vater. Clin Cancer Res 3:129–133

    CAS  PubMed  Google Scholar 

  16. Imai Y, Oda H, Tsurutani N, Nakatsuru Y, Inoue T, Ishikawa T (1997) Frequent somatic mutations of the APC and p53 genes in sporadic ampullary carcinomas. Jpn J Cancer Res 88:846–854

    CAS  PubMed  Google Scholar 

  17. Iwama T, Mishima Y, Utsunomiya J (1993) The impact of familial adenomatous polyposis on the tumorigenesis and mortality at the several organs. Its rational treatment. Ann Surg 217:101–108

    CAS  Google Scholar 

  18. Järvinen HJ, Aarnio M, Mustonen H, Aktan-Collan K, Aaltonen LA, Peltomäki P, de la Chapelle A, Mecklin JP (2000) Controlled 15-year trial on screening for colorectal cancer in families with hereditary nonpolyposis colorectal cancer. Gastroenterology 118:829–834

    Google Scholar 

  19. Kayahara M, Nagakawa T, Ohta T, Kitagawa H, Miyazaki I (1997) Surgical strategy for carcinoma of the papilla of Vater on the basis of lymphatic spreadand mode of recurrence. Surgery 121:611–617

    CAS  PubMed  Google Scholar 

  20. Kimura W, Ohtsubo K (1988) Incidence, sites of origin, and immunohistochemical and histochemical characteristics of atypical epithelium and minute carcinoma of the papilla of Vater. Cancer 61:1394–1402

    CAS  PubMed  Google Scholar 

  21. Kimura W, Futakawa N, Yamagata S, Wada Y, Kuroda A., Muto T, Esaki Y (1994) Different clinicpathologic findings in two histologic types of carcinoma of papilla of Vater. Jpn J Cancer Res 85:161–166

    CAS  PubMed  Google Scholar 

  22. Komorowski RA, Bradley K, Beggs MD, Geenan JE, Venu P (1991) Assessment of ampulla of Vater pathology. Am J Surg Pathol 15:1188–1196

    CAS  PubMed  Google Scholar 

  23. Liu TH, Chen J, Zeng XJ (1983) Histogenesis of pancreatic head and ampullary region carcinoma. Chin Med J 96:167–174

    CAS  PubMed  Google Scholar 

  24. Matsubayashi H, Watanabe H, Yamaguchi T, Ajioka Y, Nishikura K, Kijima H, Saito T (1999) Differences in mucus and K-ras mutation in relation to phenotypes of tumors of the papilla of Vater. Cancer 86:596–607

    Article  CAS  PubMed  Google Scholar 

  25. Mecklin JP, Jävinen HJ, Virolainen M (1992) The association between cholangiocarcinoma and hereditary nonpolyposis colorectal carcinoma. Cancer 69:1112–1114

    CAS  PubMed  Google Scholar 

  26. Moore PS, Orlandini S, Zamboni G, Capelli P, Rigaud G, Falconi M, Bassi C, Lemoine NR, Scarpa A (2001) Pancratic tumours: molecular pathways implicated in ductal cancer are involved in ampullary but not in exocrine nonductal or endocrine tumorigenesis. Br J Cancer 84:253–262

    Article  CAS  PubMed  Google Scholar 

  27. Noda Y, Watanabe H, Iida M, Narisawa R, Kurosaki I, Iwafuchi M, Satoh M, Ajioka Y (1992) Histologic follow-up of ampullary adenomas in patients with familial adenomatosis coli. Cancer 70:1825–1833

    CAS  PubMed  Google Scholar 

  28. Park SH, Kim Y, Park YH, Kim S, Kim YT, Kim WH (2000) Clinicopathologic correlation of p53 protein overexpression in adenoma and carcinoma of the ampulla of Vater. World J Surg 24:54–59

    Article  CAS  Google Scholar 

  29. Porschen R, Schmiegel W (1997) Prophylaxe und Früherkennung des kolorektalen Karzinoms durch endoskopische Untersuchungen. In: Sauerbruch T, Scheurlen C (Hrsg) Leitlinien der Deutschen Gesellschaft für Verdauungs- und Stoffwechselkrankheiten (DGVS). Demeter, Balingen, S 92–107

  30. Stolte M, Pscherer C (1996) Adenoma-carcinoma sequence in the papilla of Vater. Scand J Gastroenterol 31:376–382

    CAS  Google Scholar 

  31. Takashima M, Ueki T, Nagai E, Yao T, Yamaguchi K, Tanaka M, Tsuneyoshi M (2000) Carcinoma of the ampulla of Vater associated with or without adenoma: a clinicopathologic analysis of 198 cases with reference to p53 and Ki-67 immunhistochemical expressions. Mod Pathol 13:1300–1307

    CAS  PubMed  Google Scholar 

  32. Yamaguchi K, Enjoji M (1987) Carcinoma of the ampulla of Vater. Cancer 59:506–515

    CAS  PubMed  Google Scholar 

  33. Yamauchi H, Nitta A, Namiki T (1993) Carcinoma of the papilla of Vater accompaied by non-invasive adenomatous component (NAC). Tohoku J Exp Med 170:147–156

    CAS  PubMed  Google Scholar 

  34. Younes M, Riley S, Genta RM, Mosharaf M, Mody DR (2000) p53 protein accumulation in tumors of the ampulla of Vater. Cancer 76:1150–1154

    Google Scholar 

  35. Zhao B, Kimura W, Futakawa N, Muto T, Kubota K, Harihara Y, Takayama T, Makuuchi M (1999) p53 and p21/Waf1 protein expression and K-ras codon 12 mutation in carcinoma of the papilla of Vater. Am J Gastroenterol 94:2128–2134

    Google Scholar 

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Danksagung

Frau Christiane Esch und Frau Susanne Steiner sei für die Erstellung der immunhistochemischen Präparate herzlich gedankt. Herrn Gerrit Klemm danken wir für die phototechnische und digitale Bearbeitung der Abbildungen.

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  1. Pathologisches Institut, Universität Bonn

    H.-P. Fischer & H. Zhou

  2. Pathologisches Institut , Universität Bonn, Sigmund-Freud-Str. 25, 53127 , Bonn

    H.-P. Fischer

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  1. H.-P. Fischer
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  2. H. Zhou
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Correspondence to H.-P. Fischer.

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Fischer, HP., Zhou, H. Pathogenese und Histopathologie von Adenomen und Karzinomen der Papilla Vateri. Pathologe 24, 196–203 (2003). https://doi.org/10.1007/s00292-003-0617-x

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