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Zystische Pankreastumoren und ihre Klassifikation

Alte und neue Gesichter

Cystic pancreas tumors and their classification: features old and new

  • Schwerpunkt: Pankreaspathologie
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Zusammenfassung

Zystische Tumoren and tumorartige Läsionen des Pankreas sind selten. Trotzdem haben sie erhebliche Bedeutung erlangt, da sie mit den neuen radiologischen Verfahren leicht zu entdecken sind und im Gegensatz zum duktalen Adenokarzinom zumeist kurativ reseziert werden können. Durch die zunehmende Resektionsquote hat sich in den letzten Jahrzehnten auch die Kenntnis von den zystischen Pankreastumoren und ihr morphologisches Spektrum enorm erweitert. Bekannte Entitäten wurden besser charakterisiert (solid-pseudopapilläre Neoplasien, intraduktal-papillär-muzinöse Neoplasien) und neue Entitäten beschrieben (seröses oligozystisches Adenom, muzinöse nichtneoplastische Zyste, Azinuszellzystadenom und zystisches Hamartom). Diese Übersicht präsentiert die wichtigsten zystischen Tumoren und Läsionen des Pankreas, stellt eine neue Klassifikation vor und fasst die immunhistochemische Differenzialdiagnose zusammen.

Abstract

Cystic tumors and tumor-like lesions of the pancreas are rare, but have attracted a great deal of attention because they are easily recognized with new imaging methods and, in contrast to ductal adenocarcinoma, they can usually be cured surgically. The increasing resection rate in recent years has also increased our knowledge of cystic pancreatic tumors by conspicuously enlarging their morphological spectrum. Known entities have been better characterized (i.e. solid pseudopapillary neoplasm, intraductal papillary mucinous neoplasm) and new ones described (serous oligocystic adenoma, mucinous non-neoplastic cyst, acinar cell cystadenoma and cystic hamartoma). This review discusses the most important cystic tumors and tumor-like lesions, presents a new classification, and summarizes the immunohistochemical differential diagnosis.

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Literatur

  1. Adsay NV, Adair CF, Heffess CS, Klimstra DS (1996) Intraductal oncocytic papillary neoplasms of the pancreas. Am J Surg Pathol 20: 980–994

    Google Scholar 

  2. Adsay NV, Hasteh F, Cheng JD et al. (2002) Lymphoepithelial cysts of the pancreas: a report of 12 cases and a review of the literature. Mod Pathol 15: 492–501

    Article  PubMed  Google Scholar 

  3. Adsay NV, Klimstra DS (2000) Cystic lesions of the pancreas. Saunders, Philadelphia

  4. Adsay NV, Klimstra DS, Compton CC (2000) Cystic lesions of the pancreas. Introduction. Semin Diagn Pathol 17: 1–6

    Google Scholar 

  5. Adsay NV, Longnecker DS, Klimstra DS (2000) Pancreatic tumors with cystic dilatation of the ducts: intraductal papillary mucinous neoplasms and intraductal oncocytic papillary neoplasms. Semin Diagn Pathol 17: 16–30

    CAS  PubMed  Google Scholar 

  6. Albores-Saavedra J (2002) Acinar cystadenoma of the pancreas: a previously undescribed tumor. Ann Diagn Pathol 6: 113–115

    Google Scholar 

  7. Albores-Saavedra J, Gould EW, Angeles-Angeles A, Henson DE (1990) Cystic tumors of the pancreas. In: PP Rosen, Fechner RE (eds) Pathology Annual, vol 25, part 2. Appleton & Lange, East Norwalk, pp 19–50

  8. Capella C, Solcia E, Klöppel G, Hruban RH (2000) Serous cystic neoplasms of the pancreas. In: Hamilton SR, Aaltonen LA (eds) Pathology and genetics of tumours of the digestive system. WHO classification of tumours. IARC Press, Lyon, pp 231–233

  9. Casadei R, Campione O, Greco VM, Marrano D (1996) Congenital true pancreatic cysts in young adults: case report and literature review. Pancreas 12: 419–421

    Google Scholar 

  10. Chatelain D, Paye F, Mourra N, Scoazec JY, Baudrimont M, Parc R, Fléjou JF (2002) Unilocular acinar cell cystadenoma of the pancreas an unusual acinar cell tumor. Am J Clin Pathol 118: 211–214

    Google Scholar 

  11. Compagno J, Oertel JE (1978) Microcystic adenomas of the pancreas (glycogen-rich cystadenomas): a clinicopathologic study of 34 cases. Am J Clin Pathol 69: 289–298

    Google Scholar 

  12. Compagno J, Oertel JE (1978) Mucinous cystic neoplasms of the pancreas with overt and latent malignancy (cystadenocarcinoma and cystadenoma). A clinicopathologic study of 41 cases. Am J Clin Pathol 69: 573–580

    CAS  PubMed  Google Scholar 

  13. Compton CC (2000) Serous cystic tumors of the pancreas. Semin Diagn Pathol 17: 43–55

    Google Scholar 

  14. Egawa N, Maillet B, Schröder S, Foulis A, Mukai K, Klöppel G (1994) Serous oligocystic and ill-demarcated adenoma of the pancreas: a variant of serous cystic adenoma. Virchows Arch 424: 13–17

    Google Scholar 

  15. Flaherty MJ, Benjamin DR (1992) Multicystic pancreatic hamartoma: a distinctive lesion with immunohistochemical and ultrastructural study. Hum Pathol 23: 1309–1312

    Article  CAS  PubMed  Google Scholar 

  16. Fléjou JF, Potet F, Molas G, Bernades P, Amouyal P, Fékété F (1993) Cystic dystrophy of the gastric and duodenal wall developing in heterotopic pancreas: an unrecognized entity. Gut 34: 343–347

    PubMed  Google Scholar 

  17. Fukushima N, Mukai K, Sakamoto M, Shimada K, Kosuge T, Kinoshita T, Hirohashi S (2001) Invasive carcinoma derived from intraductal papillary-mucinous carcinoma of the pancreas: clinicopathologic and immunohistochemical study of 8 cases. Virchows Arch 439: 6–13

    Google Scholar 

  18. George DH, Murphy F, Michalski R, Ulmer BG (1989) Serous cystadenocarcinoma of the pancreas: a new entity? Am J Surg Pathol 13: 61–66

    Google Scholar 

  19. Hamilton SR, Aaltonen LA (2000) Pathology and genetics of tumours of the digestive system. WHO classification of tumours. IARC Press, Lyon

  20. Hastings PR, Nance FC, Becker WF (1975) Changing patterns in the management of pancreatic pseudocysts. Ann Surg 181: 546–551

    Google Scholar 

  21. Healy DL, Burger HG, Mamers P et al. (1993) Elevated serum inhibin concentrations in postmenopausal women with ovarian tumors. N Engl J Med 329: 1539–1542

    Google Scholar 

  22. Hodgkinson DJ, ReMine WH, Weiland LH (1978) A clinicopathologic study of 21 cases of pancreatic cystadenocarcinoma. Ann Surg 188: 679–684

    Google Scholar 

  23. Klöppel G (1998) Clinicopathologic view of intraductal papillary-mucinous tumor of the pancreas. Hepatogastroenterol 45: 1981–1985

    Google Scholar 

  24. Klöppel G (2000) Pseudocysts and other non-neoplastic cysts of the pancreas. Semin Diagn Pathol 17: 7–15

    Google Scholar 

  25. Klöppel G, Hruban RH, Longnecker DS, Adler G, Kern SE, Partanen TJ (2000) Ductal adenocarcinoma of the pancreas. In: Hamilton SR, Aaltonen LA (eds) Pathology and genetics of tumours of the digestive system. WHO classification of tumours. IARC Press, Lyon, pp 221–230

  26. Klöppel G, Lüttges J, Klimstra D, Hruban R, Kern S, Adler G (2000) Solid-pseudopapillary neoplasm. In: Hamilton SR, Aaltonen LA (eds) Pathology and genetics of tumours of the digestive system. WHO classification of tumours. IARC Press, Lyon, pp 246–248

  27. Klöppel G, Solcia E, Longnecker DS, Capella C, Sobin LH (1996) Histological typing of tumours of the exocrine pancreas. Second edition. WHO international histological classification of tumours. Springer, Berlin Heidelberg New Aork Tokio

  28. Kosmahl M, Egawa N, Schröder S, Carneiro F, Lüttges J, Klöppel G (2002) Mucinous nonneoplastic cyst of the pancreas: a novel nonneoplastic cystic change? Mod Pathol 15: 154–158

    Google Scholar 

  29. Kosmahl M, Pauser U, Peters K, Sipos B, Lüttges J, Kremer B, Klöppel G (2004) Cystic neoplasms of the pancreas and tumor-like lesions with cystic features: a review of 418 cases and a classification proposal. Virchows Arch 445: 168–178

    Article  CAS  PubMed  Google Scholar 

  30. Kosmahl M, Seada LS, Jänig U, Harms D, Klöppel G (2000) Solid-pseudopapillary tumor of the pancreas: its origin revisited. Virchows Arch 436: 473–480

    Article  CAS  PubMed  Google Scholar 

  31. Kosmahl M, Wagner J, Peters K, Sipos B, Klöppel G (2004) Serous cystic neoplasms of the pancreas: an immunohistochemical analysis revealing alpha-inhibin, neuron-specific enolase, and MUC6 as new markers. Am J Surg Pathol 28: 339–346

    Google Scholar 

  32. Lewandrowski K, Warshaw A, Compton C (1992) Macrocystic serous cystadenoma of the pancreas: a morphologic variant differing from microcystic adenoma. Hum Pathol 23: 871–875

    Google Scholar 

  33. Longnecker DS, Adler G, Hruban RH, Klöppel G (2000) Intraductal papillary-mucinous neoplasms of the pancreas. In: Hamilton SR, Aaltonen LA (eds) Pathology and genetics of tumours of the digestive system. WHO classification of tumours. IARC Press, Lyon, pp 237–240

  34. Lüttges J, Feyerabend B, Buchelt T, Pacena M, Klöppel G (2002) The mucin profile of noninvasive and invasive mucinous cystic neoplasms of the pancreas. Am J Surg Pathol 26: 466–471

    Google Scholar 

  35. Moore PS, Zamboni G, Brighenti A et al. (2001) Molecular characterization of pancreatic serous microcytic adenomas. Evidence for a tumor suppressor gene on chromosome 10q. Am J Pathol 158: 317–321

    Google Scholar 

  36. Morohoshi T, Hamamoto T, Kunimara T et al. (1991) Epidermoid cyst derived from an accessory spleen in the pancreas. A case report with literature survey. Acta Pathol Jpn 41: 916–921

    Google Scholar 

  37. Morohoshi T, Kanda M, Asanuma K, Klöppel G (1989) Intraductal papillary neoplasms of the pancreas. A clinicopathologic study of six patients. Cancer 64: 1329–1335

    Google Scholar 

  38. Ohhashi K, Murakami F, Takekoshi T et al. (1982) Four cases of „mucin-producing“ cancer of the pancreas on specific findings of the papilla of Vater (Japanese). Proc Dig Endosc 20: 348

    Google Scholar 

  39. Paal E, Thompson LD, Heffess CS (1998) A clinicopathologic and immunohistochemical study of ten pancreatic lymphangiomas and a review of the literature. Cancer 82: 2150–2158

    Article  CAS  PubMed  Google Scholar 

  40. Pappas S, Diaz L, Talamonti M (2002) A cystic pancreatic mass discovered in a patient with ileocecal carcinoid (Pathologic quiz case). Arch Pathol Lab Med 126: 229–230

    Google Scholar 

  41. Perez-Ordonez B, Naseem A, Lieberman PH, Klimstra DS (1996) Solid serous adenoma of the pancreas. The solid variant of serous cystadenoma? Am J Surg Pathol 20: 1401–1405

    Google Scholar 

  42. Pilcher CS, Bradley ELI, Majmudar B (1982) Enterogenous cyst of the pancreas. Am J Gastroenterol 77: 576–577

    Google Scholar 

  43. Rattner DW, Fernandez-del Castillo C, Warshaw AL (1999) Cystic pancreatic neoplasms. Ann Oncol 10 Suppl.: S104-S106

  44. Rickaert F, Cremer M, Devière J et al. (1991) Intraductal mucin-hypersecreting neoplasms of the pancreas. A clinicopathologic study of eight patients. Gastroenterology 101: 512–519

    Google Scholar 

  45. Sessa F, Bonato M, Frigerio B et al. (1990) Ductal cancers of the pancreas frequently express markers of gastrointestinal epithelial cells. Gastroenterology 98: 1655–1665

    Google Scholar 

  46. Sessa F, Solcia E, Capella C et al. (1994) Intraductal papillary-mucinous tumours represent a distinct group of pancreatic neoplasms: an investigation of tumour cell differentiation and K-ras, p53, and c-erbB-2 abnormalities in 26 patients. Virchows Arch 425: 357–367

    Article  CAS  PubMed  Google Scholar 

  47. Solcia E, Capella C, Klöppel G (1997) Tumors of the pancreas. AFIP Atlas of Tumor Pathology, third series, fascicle 20. Armed Forces Institute of Pathology, Washington, D.C.

  48. Sumiyoshi Y, Yamashita Y, Maekawa T, Sakai T, Shirakusa T (2000) A case of hemorrhagic cyst of the pancreas resembling the cystic endometriosis. Int Surg 85: 67–70

    Google Scholar 

  49. Terris B, Ponsot T, Paye F et al. (2000) Intraductal papillary mucinous tumors of the pancreas confined to secondary ducts show less aggressive pathologic features as compared with those involving the main pancreatic duct. Am J Surg Pathol 24: 1372–1377

    Google Scholar 

  50. Thompson LD, Becker RC, Przygodzki RM, Adair CF, Heffess CS (1999) Mucinous cystic neoplasm (mucinous cystadenocarcinoma of low-grade malignant potential) of the pancreas: a clinicopathologic study of 130 cases. Am J Surg Pathol 23: 1–16

    Article  CAS  PubMed  Google Scholar 

  51. Verbeke C, Harle M, Sturm J (1996) Cystic endometriosis of the upper abdominal organs. Report on three cases and review of the literature. Pathol Res Pract 192: 300–304

    Google Scholar 

  52. Wilentz RE, Albores-Saavedra J, Hruban RH (2000) Mucinous cystic neoplasms of the pancreas. Semin Diagn Pathol 17: 31–42

    CAS  PubMed  Google Scholar 

  53. Wilentz RE, Albores-Saavedra J, Zahurak M, Talamini MA, Yeo CJ, Cameron JL, Hruban RH (1999) Pathologic examination accurately predicts prognosis in mucinous cystic neoplasms of the pancreas. Am J Surg Pathol 23: 1320–1327

    Google Scholar 

  54. Wouters K, Ectors N, Van Steenbergen W, Aerts R, Driessen A, Van Hoe L, Geboes K (1998) A pancreatic mucinous cystadenoma in a man with mesenchymal stroma, expressing oestrogen and progesterone receptors. Virchows Arch 432: 187–189

    Google Scholar 

  55. Yorganci K, Iret D, Sayek I (2000) A case of primary hydatid disease of the pancreas simulating cystic neoplasm. Pancreas 21: 104–105

    Google Scholar 

  56. Zamboni G, Klöppel G, Hruban RH, Longnecker DS, Adler G (2000) Mucinous cystic neoplasms of the pancreas. In: Hamilton SR, Aaltonen LA (eds) Pathology and genetics of tumours of the digestive system. WHO classification of tumours. IARC Press, Lyon, pp 234–236

  57. Zamboni G, Scarpa A, Bogina G et al. (1999) Mucinous cystic tumors of the pancreas. Clinicopathological features, prognosis and relationship to other mucinous cystic tumors. Am J Surg Pathol 23: 410–422

    Article  CAS  PubMed  Google Scholar 

  58. Zamboni G, Terris B, Scarpa A et al. (2002) Acinar cell cystadenoma of the pancreas. A new entity? Am J Surg Pathol 26: 698–704

    Google Scholar 

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Kosmahl, M., Pauser, U., Anlauf, M. et al. Zystische Pankreastumoren und ihre Klassifikation. Pathologe 26, 22–30 (2005). https://doi.org/10.1007/s00292-004-0734-1

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