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Cystic neoplasms of the pancreas and tumor-like lesions with cystic features: a review of 418 cases and a classification proposal

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Abstract

Although cystic neoplasms and lesions of the pancreas are rare, they have attracted a great deal of attention because of their potential curability. Since, in recent years, several new entities have been identified, the relative frequency of the tumors and their classification need to be reevaluated. In a series of 1454 tumorous lesions of the pancreas collected between 1971 and 2003 in our surgical pathology files and consultation files, all cystic pancreatic neoplasms and tumor-like lesions were identified and typed both histologically and immunohistochemically. There were 418 cases (29%) showing cysts with a diameter ranging between 0.5 cm and 27 cm. Most common were solid pseudopapillary neoplasms (21%) and intraductal papillary-mucinous neoplasms (18%). When only the cystic neoplasms and lesions that had been resected in a single institution were considered, intraductal papillary mucinous neoplasms were the most frequent cystic neoplasms, while solid pseudopapillary neoplasms took fifth place behind ductal adenocarcinomas with cystic features, serous cystic neoplasms and mucinous cystic neoplasms. The most frequent cystic tumor-like lesions were pancreatitis-associated pseudocysts. New and rare entities that have recently been identified are mucinous nonneoplastic cysts, acinar cell cystadenomas and cystic hamartomas. Bearing in mind that figures from referral centers such as ours may be biased regarding the relative frequency of lesions, we concluded from our data that intraductal papillary-mucinous neoplasms are the most frequently occurring pancreatic cystic neoplasms, rather than solid pseudopapillary neoplasms. It was possible to classify all cystic lesions encountered in our files or described in the literature in a new system that distinguishes between neoplastic and nonneoplastic lesions, with further subdivisions into epithelial (adenomas, borderline neoplasms and carcinomas) and nonepithelial tumors. This classification is easy to handle and enables a distinction on the basis of clinical behavior and prognosis.

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References

  1. Adsay NV, Klimstra DS (2000) Cystic forms of typically solid pancreatic tumors. Semin Diagn Pathol 17:81–88

    CAS  PubMed  Google Scholar 

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

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

    Article  CAS  PubMed  Google Scholar 

  4. Adsay NV, Hasteh F, Cheng JD, Klimstra DS (2000) Squamous-lined cysts of the pancreas: lymphoepithelial cysts, dermoid cysts (teratomas) and accessory-splenic epidermoid cysts. Semin Diagn Pathol 17:56–65

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  6. Adsay NV, Hasteh F, Cheng JD, Bejarano PA, Lauwers GY, Batts KP, Klöppel G, Klimstra DS (2002) Lymphoepithelial cysts of the pancreas: a report of 12 cases and a review of the literature. Mod Pathol 15:492–501

    PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  8. Bernd HW, Burmester E, Horny HP (2002) Unusual coincidence of lymphoepithelial cyst and mucinous cystadenoma of the pancreas. Virchows Arch 441:303–305

    Article  CAS  PubMed  Google Scholar 

  9. Brown SZ, Owen DA, O’Connell JX, Scudamore CH (1998) Schwannoma of the pancreas: a report of two cases and a review of the literature. Mod Pathol 11:1178–1182

    CAS  PubMed  Google Scholar 

  10. Cantrell BB, Cubilla AL, Erlandson RA, Fortner J, Fitzgerald PJ (1981) Acinar cell cystadenocarcinoma of human pancreas. Cancer 47:410–416

    CAS  PubMed  Google Scholar 

  11. 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

  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. Cubilla AL, Fitzgerald PJ (1984) Tumors of the exocrine pancreas. AFIP atlas of tumor pathology, 2nd series, fascicle 19. Armed Forces Institute of Pathology, Washington, DC

  14. de Lima JE, Javitt MC, Mathur SC (1999) Mucinous cystic neoplasm of the pancreas. Radiographics 19:807–811

    PubMed  Google Scholar 

  15. 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

    CAS  PubMed  Google Scholar 

  16. Frantz VK (1959) Tumors of the pancreas. AFIP atlas of tumor pathology, 1st series, section 7, fascicles 27 and 28. Armed Forces Institute of Pathology, Washington, DC

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

    CAS  PubMed  Google Scholar 

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

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

    CAS  PubMed  Google Scholar 

  20. Hoorens A, Lemoine NR, McLellan E, Morohoshi T, Kamisawa T, Heitz PU, Stamm B, Rüschoff J, Wiedenmann B, Klöppel G (1993) Pancreatic acinar cell carcinoma. An analysis of cell lineage markers, p53 expression, and Ki-ras mutation. Am J Pathol 143:685–698

    CAS  PubMed  Google Scholar 

  21. Hruban RH, Takaori K, Klimstra DS, Adsay NV, Albores-Saavedra J, Biankin AV, Biankin SA, Compton C, Fukushima N, Furukawa T, Goggins M, Kato Y, Klöppel G, Longnecker DS, Lüttges J, Maitra A, Offerhaus GJA, Shimizu M, Yonezawa S (2004) An illustrated consensus on the classification of pancreatic intraepithelial neoplasia (PanIN) and intraductal papillary mucinous neoplasms (IPMNs). Am J Surg Pathol (in press)

  22. Jaffe R (1992) The pancreas. In: Stocker JT, Dehner LP (eds) Pediatric pathology, Vol. 2. Lippincott, Philadelphia, PA, pp 791–823

  23. Kattan YB (1975) Hydatid cysts in pancreas. BMJ 4:729–730

    CAS  Google Scholar 

  24. Klimstra DS, Wenig BM, Heffess CS (2000) Solid-pseudopapillary tumor of the pancreas: a typically cystic carcinoma of low malignant potential. Semin Diagn Pathol 17:66–80

    CAS  PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  26. Klöppel G, Kosmahl M (2001) Cystic lesions and neoplasms of the pancreas. The features are becoming clearer. Pancreatology 1:648–655

    Article  CAS  PubMed  Google Scholar 

  27. Klöppel G, Morohoshi T, John HD, Oehmichen W, Opitz K, Angelkort A, Lietz H, Rückert K (1981) Solid and cystic acinar cell tumour of the pancreas. A tumour in young women with favourable prognosis. Virchows Arch 392:171–183

    Google Scholar 

  28. Klöppel G, Solcia E, Longnecker DS, Capella C, Sobin LH (1996) Histological typing of tumours of the exocrine pancreas, 2nd edn. WHO International histological classification of tumours. Springer, Berlin Heidelberg New York

  29. 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

  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

    CAS  PubMed  Google Scholar 

  31. 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

    PubMed  Google Scholar 

  32. 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

    PubMed  Google Scholar 

  33. Lavine JE, Harrison M, Heyman MB (1989) Gastrointestinal duplications causing relapsing pancreatitis in children. Gastroenterology 97:1556–1558

    CAS  PubMed  Google Scholar 

  34. le Borgne J, de Calan L, Partensky C, French Surgical Association (1999) Cystadenomas and cystadenocarcinomas of the pancreas: a multiinstitutional retrospective study of 398 cases. Ann Surg 230:152–161

    PubMed  Google Scholar 

  35. Lee JS, Kim HS, Jung JJ, Han SW, Kim YB (2001) Ancient schwannoma of the pancreas mimicking a cystic tumor. Virchows Arch 439:697–699

    CAS  PubMed  Google Scholar 

  36. 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

    CAS  PubMed  Google Scholar 

  37. Ligneau B, Lombard-Bohas C, Partensky C, Valette PJ, Calender A, Dumortier J, Gouysse G, Boulez J, Napoleon B, Berger F, Chayvialle JA, Scoazec JY (2001) Cystic endocrine tumors of the pancreas: clinical, radiologic, and histopathologic features in 13 cases. Am J Surg Pathol 25:760

    Article  Google Scholar 

  38. Liu TH, Consorti ET (2000) Inflammatory pseudotumor presenting as a cystic tumor of the pancreas. Am Surg 66:993–997

    CAS  PubMed  Google Scholar 

  39. 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

  40. Luchtrath H, Schriefers KH (1985) A pancreatic cyst with features of a so-called branchiogenic cyst (in German). Pathologe 6:217–219

    CAS  PubMed  Google Scholar 

  41. Lüttges J, Pierré E, Zamboni G, Weh G, Lietz H, Kussmann J, Klöppel G (1997) Maligne nicht-epitheliale Tumoren des Pankreas. Pathologe 18:233–237

    Article  PubMed  Google Scholar 

  42. Lüttges J, Zamboni G, Longnecker D, Klöppel G (2001) The immunohistochemical mucin expression pattern distinguishes different types of intraductal papillary mucinous neoplasms of the pancreas and determines their relationship to mucinous noncystic carcinoma and ductal adenocarcinoma. Am J Surg Pathol 25:942–948

    PubMed  Google Scholar 

  43. 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

    Article  CAS  PubMed  Google Scholar 

  44. Mahmood K, Butt MM, Haleem A (1989) Duplication of the duodenum exhibiting heterotopia in the pancreas: report of a case. Ann Saudi Med 9:602–604

    Google Scholar 

  45. Mares AJ, Hirsch M (1977) Congenital cysts of the head of the pancreas. J Pediatr Surg 12:547–552

    Article  CAS  PubMed  Google Scholar 

  46. Miles RM (1959) Pancreatic cyst in the newborn. Ann Surg 149:576–581

    CAS  PubMed  Google Scholar 

  47. 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

    CAS  PubMed  Google Scholar 

  48. Oehler U, Jurs M, Klöppel G, Helpap B (1997) Osteoclast-like giant cell tumour of the pancreas presenting as a pseudocyst-like lesion. Virchows Arch 431:215–218

    Article  CAS  PubMed  Google Scholar 

  49. 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 (in Japanese). Proc Dig Endosc 20:348

    Google Scholar 

  50. Pasternack A, Hjelt L (1961) Cystic disease of the kidneys, liver, and pancreas. Ann Paediatr Finnlandiae 7:138–145

    CAS  Google Scholar 

  51. 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

    Article  CAS  PubMed  Google Scholar 

  52. Rickaert F, Cremer M, Devière J, Tavares L, Lambilliotte JP, Schröder S, Wurbs D, Klöppel G (1991) Intraductal mucin-hypersecreting neoplasms of the pancreas. A clinicopathologic study of eight patients. Gastroenterology 101:512–519

    CAS  PubMed  Google Scholar 

  53. Schmidt-Rohlfing B, Siech M, Mattfeldt T, Schoenberg MH, Beger HG (1998) Zystische Neoplasien des Pankreas: operative Therapie und Heilungschancen. Z Gastroenterol 36:939–945

    CAS  PubMed  Google Scholar 

  54. Sessa F, Solcia E, Capella C, Bonato M, Scarpa A, Zamboni G, Pellegata NS, Ranzani GN, Rickaert F, Klöppel G (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

    CAS  PubMed  Google Scholar 

  55. 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, DC

  56. Stamm B, Burger H, Hollinger A (1987) Acinar cell cystadenocarcinoma of the pancreas. Cancer 60:2542–2547

    CAS  PubMed  Google Scholar 

  57. Talamini MA, Pitt HA, Hruban RH, Boitnott JK, Coleman J, Cameron JL (1992) Spectrum of cystic tumors of the pancreas. Am J Surg 163:117–123

    Article  CAS  PubMed  Google Scholar 

  58. Tan G, Vitellas K, Morrison C, Frankel WL (2003) Cystic schwannoma of the pancreas. Ann Diagn Pathol 7:285–291

    Article  PubMed  Google Scholar 

  59. Tilger A (1894) Beitrag zur pathologischen Anatomie und Aetiologie der Pankreas-Cysten. Arch Pathol Anat Physiol Klin Med 137:348–375

    Google Scholar 

  60. Wegelin C (1921) Zur Genese und Einteilung der Pankreaszysten. Verh Dtsch Pathol Ges 169–173

  61. Widmaier J, Mattfeldt T, Siech M, Beger HG (1996) Serous cystadenocarcinoma of the pancreas. Int J Pancreatol 20:135–139

    CAS  PubMed  Google Scholar 

  62. Yamane M (1921) Beiträge zur Kenntnis der Pankreaszysten. Akad. Buchhandl. Bern: Paul Haupt, Bern

  63. Zamboni G, Scarpa A, Bogina G, Iacono C, Bassi C, Talamini G, Sessa F, Capella C, Solcia E, Rickaert F, Mariuzzi GM, Klöppel G (1999) Mucinous cystic tumors of the pancreas. Clinicopathological features, prognosis and relationship to other mucinous cystic tumors. Am J Surg Pathol 23:410–422

    CAS  PubMed  Google Scholar 

  64. Zamboni G, Terris B, Scarpa A, Kosmahl M, Capelli P, Klimstra DS, Lam PWY, Klöppel G (2002) Acinar cell cystadenoma of the pancreas. A new entity? Am J Surg Pathol 26:698–704

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Pathology, University of Kiel, Germany

    M. Kosmahl, U. Pauser, K. Peters, B. Sipos, J. Lüttges & G. Klöppel

  2. Department of Surgery, University of Kiel, Germany

    B. Kremer

  3. Department of Pathology, University of Kiel, Michaelisstrasse 11, 24105 , Kiel, Germany

    M. Kosmahl

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  1. M. Kosmahl
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  2. U. Pauser
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  3. K. Peters
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Kosmahl, M., Pauser, U., Peters, K. et al. 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 (2004). https://doi.org/10.1007/s00428-004-1043-z

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  • DOI: https://doi.org/10.1007/s00428-004-1043-z

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