Advertisement

Virchows Archiv

, Volume 458, Issue 5, pp 537–546 | Cite as

Primary neuroendocrine tumors of the main pancreatic duct: a rare entity

  • Thomas Walter
  • Valérie Hervieu
  • Mustapha Adham
  • Rodica Gincul
  • Gilles Poncet
  • Frank Pilleul
  • Jean-Alain Chayvialle
  • Christian Partensky
  • Catherine Lombard-Bohas
  • Jean-Yves ScoazecEmail author
Original Article

Abstract

Very few cases of primary neuroendocrine tumors of the main pancreatic duct have been reported. This paucity has hampered an accurate description of the distinctive clinical and pathological features of these tumors and the correct evaluation of the diagnostic and therapeutic problems which they may raise. We report here five additional cases in order to underline the clinical, histological, and immunohistochemical features of this tumor entity. There were three male and two female, aged 43–72 years; in all patients, but one, who presented with epigastric pain, the diagnosis was made after the incidental discovery of a dilatation of the main pancreatic duct. The preoperative diagnosis was ductal adenocarcinoma in one case, IPMN in one case and neoplastic stenosis of unknown etiology in four cases. Surgical resection was performed in all cases. The diagnosis of neuroendocrine tumor was made at histological examination. All lesions were small, ranging from 5 to 15 mm. They had a predominantly intramural growth. The growth pattern was nodular in three cases, circumferential in two; there was no intra-luminal component. All cases were well-differentiated neuroendocrine neoplasms of low histological grade (G1); four cases expressed serotonin. One case was associated with regional lymph node metastases. All cases were cured by surgery alone; no recurrence was observed at the end of the follow-up period. In conclusion, despite their rarity, primary neuroendocrine tumors of the main pancreatic duct deserve recognition and must be considered in the etiological diagnosis of ductal stenosis.

Keywords

Gastroenteropancreatic neuroendocrine tumors Main pancreatic duct Serotonin Carcinoid tumors 

Notes

Conflict of interest statement

We declare that we have no conflict of interest.

References

  1. 1.
    Modlin IM, Oberg K, Chung DC, Jensen RT, de Herder WW, Thakker RV, Caplin M, Delle Fave G, Kaltsas GA, Krenning EP, Moss SF, Nilsson O, Rindi G, Salazar R, Ruszniewski P, Sundin A (2008) Gastroenteropancreatic neuroendocrine tumours. Lancet Oncol 9:61–72PubMedCrossRefGoogle Scholar
  2. 2.
    Davies K, Conlon KC (2009) Neuroendocrine tumors of the pancreas. Curr Gastroenterol Rep 11:119–127PubMedCrossRefGoogle Scholar
  3. 3.
    Capelli P, Martignoni G, Pedica F, Falconi M, Antonello D, Malpeli G, Scarpa A (2009) Endocrine neoplasms of the pancreas: pathologic and genetic features. Arch Pathol Lab Med 133:350–364PubMedGoogle Scholar
  4. 4.
    Falconi M, Plockinger U, Kwekkeboom DJ, Manfredi R, Korner M, Kvols L, Pape UF, Ricke J, Goretzki PE, Wildi S, Steinmuller T, Oberg K, Scoazec JY (2010) Well-differentiated pancreatic nonfunctioning tumors/carcinoma. Neuroendocrinology 84:196–211CrossRefGoogle Scholar
  5. 5.
    Obara T, Shudo R, Fujii T, Tanno S, Mizukami Y, Izawa T, Saitoh Y, Satou S, Kohgo Y (2000) Pancreatic duct obstruction caused by malignant islet cell tumors of the pancreas. Gastrointest Endosc 51:604–607PubMedCrossRefGoogle Scholar
  6. 6.
    Sarles H, Cambon P, Choux R, Payan MJ, Odaira S, Laugier R, Sahel J (1988) Chronic obstructive pancreatitis due to tiny (0.6 to 8 mm) benign tumors obstructing pancreatic ducts: report of three cases. Pancreas 3:232–237PubMedCrossRefGoogle Scholar
  7. 7.
    Nagai E, Yamaguchi K, Hashimoto H, Sakurai T (1992) Carcinoid tumor of the pancreas with obstructive pancreatitis. Am J Gastroenterol 87:361–364PubMedGoogle Scholar
  8. 8.
    Dei Tos AP, Laurino L, De Boni M, Doglioni C (1993) Preoperative cytodiagnosis of primitive carcinoid tumor of the Wirsung duct: a case report with immunocytochemical study. Diagn Cytopathol 9:471–474PubMedCrossRefGoogle Scholar
  9. 9.
    Kim HC, Park SI, Park SJ, Shin HC, Oh MH, Kim CH, Kim TY, Kim HH, Bae WK, Kim IY (2005) Pancreatic carcinoid tumor with obstructive pancreatitis: multislice helical CT appearance: case report. Abdom Imaging 30:601–604PubMedCrossRefGoogle Scholar
  10. 10.
    Hamada Y, Nakayama Y, Maeshiro K, Ikeda T, Hayashi H, Iwasaki H (2009) Two cases of primary carcinoid tumor of the pancreas associated with marked stenosis of the main pancreatic duct. Pancreas 38:834–835PubMedCrossRefGoogle Scholar
  11. 11.
    Rindi G, Kloppel G, Alhman H, Caplin M, Couvelard A, de Herder WW, Erikssson B, Falchetti A, Falconi M, Komminoth P, Korner M, Lopes JM, McNicol AM, Nilsson O, Perren A, Scarpa A, Scoazec JY, Wiedenmann B (2006) TNM staging of foregut (neuro)endocrine tumors: a consensus proposal including a grading system. Virchows Arch 449:395–401PubMedCrossRefGoogle Scholar
  12. 12.
    Solcia E, Klöppel G, Sobin L (2000) Histological typing of endocrine tumours, 2nd edn. Springer, New YorkGoogle Scholar
  13. 13.
    Rindi G, Klimstra DS, Arnold R, Klöppel G, Bosman FT, Komminoth P, Capella C, Solcia E (2010) Nomenclature and classification of neuroendocrine neoplasms of the digestive system. In: Bosman FT, Carneiro F, Hruban RH, Theise ND (eds) WHO classification of tumours of the digestive system. IARC, Lyon, pp 13–14Google Scholar
  14. 14.
    Sobin LH, Gospodarowicz MK, Wittekind C (2009) TNM classification of malignant tumors, 7th edn. Wiley, New YorkGoogle Scholar
  15. 15.
    Odaira C, Choux R, Payan MJ, Bockman DE, Sarles H (1987) Chronic obstructive pancreatitis, nesidioblastosis, and small endocrine pancreatic tumor. Dig Dis Sci 32:770–774PubMedCrossRefGoogle Scholar
  16. 16.
    Jukemura J, Montagnini AL, Perini MV, de Almeida JL, Rodrigues JJ, da Cunha JE (2006) Acute pancreatitis associated with neuroendocrine tumor of the pancreas. JOP 7:56–61PubMedGoogle Scholar
  17. 17.
    Uemura K, Murakami Y, Hayashidani Y, Sudo T, Hashimoto Y, Ohge H, Sasaki T, Arihiro K, Sueda T (2009) Main pancreatic duct obstruction due to a small nonfunctioning endocrine tumor of the pancreas. Hiroshima J Med Sci 58:45–48PubMedGoogle Scholar
  18. 18.
    Simpson WF, Adams DB, Metcalf JS, Anderson MC (1988) Nonfunctioning pancreatic neuroendocrine tumors presenting as pancreatitis: report of four cases. Pancreas 3:223–231PubMedCrossRefGoogle Scholar
  19. 19.
    Pech O, Lingenfelser T, Wunsch P (2000) Pancreatic glucagonoma as a rare cause of chronic obstructive pancreatitis. Gastrointest Endosc 52:562–564PubMedCrossRefGoogle Scholar
  20. 20.
    Ramsay D, Gibson P, Edmunds S, Mendelson R (2001) Pancreatic islet cell tumours presenting as recurrent acute pancreatitis: imaging features in three cases. Australas Radiol 45:520–523PubMedCrossRefGoogle Scholar
  21. 21.
    Azumi Y, Isaji S, Kato H, Kuriyama N, Kishiwada M, Hamada T, Mizuno S, Usui M, Sakurai H, Tabata M, Inoue H, Katsurahara M, Takei Y, Uchida K, Shiraishi T (2009) A case of a tiny (1.2 cm) non-functioning pancreatic endocrine tumor invading the main pancreatic duct successfully treated by laparoscopy-assisted distal pancreatectomy with splenectomy. Suizo 24:717–725CrossRefGoogle Scholar
  22. 22.
    Shimizu K, Shiratori K, Toki F, Suzuki M, Imaizumi T, Takasaki K, Kobayashi M, Hayashi N (1999) Nonfunctioning islet cell tumor with a unique pattern of tumor growth. Dig Dis Sci 44:547–551PubMedCrossRefGoogle Scholar
  23. 23.
    Kitami CE, Shimizu T, Sato O, Kurosaki I, Mori S, Yanagisawa Y, Ajioka Y, Hatakeyama K (2000) Malignant islet cell tumor projecting into the main pancreatic duct. J Hepatobiliary Pancreat Surg 7:529–533PubMedCrossRefGoogle Scholar
  24. 24.
    Terada T, Kawaguchi M, Furukawa K, Sekido Y, Osamura Y (2002) Minute mixed ductal-endocrine carcinoma of the pancreas with predominant intraductal growth. Pathol Int 52:740–746PubMedCrossRefGoogle Scholar
  25. 25.
    Akatsu T, Wakabayashi G, Aiura K, Suganuma K, Takigawa Y, Wada M, Kawachi S, Tanabe M, Ueda M, Shimazu M, Sakamoto M, Kitajima M (2004) Intraductal growth of a nonfunctioning endocrine tumor of the pancreas. J Gastroenterol 39:584–588PubMedGoogle Scholar
  26. 26.
    Kawakami H, Kuwatani M, Hirano S, Kondo S, Nakanishi Y, Itoh T, Asaka M (2007) Pancreatic endocrine tumors with intraductal growth into the main pancreatic duct and tumor thrombus within the portal vein: a case report and review of the literature. Intern Med 46:273–277PubMedCrossRefGoogle Scholar
  27. 27.
    Inagaki M, Watanabe K, Yoshikawa D, Suzuki S, Ishizaki A, Matsumoto K, Haneda M, Tokusashi Y, Miyokawa N, Sato S, Kasai S (2007) A malignant nonfunctioning pancreatic endocrine tumor with a unique pattern of intraductal growth. J Hepatobiliary Pancreat Surg 14:318–323PubMedCrossRefGoogle Scholar
  28. 28.
    Eto K, Kawakami H, Kuwatani M, Haba S, Hirano S, Kondo S, Kubota K, Matuno Y, Hirayama A, Gotouda Y, Asaka M (2009) A malignant pancreatic neuro-endocrine tumor with intraductal growth within the main pancreatic duct and tumor thrombus within the splenic vein. Suizo 24:537–547CrossRefGoogle Scholar
  29. 29.
    Fassan M, Pizzi S, Pasquali C, Parenti AR (2009) Pancreatic endocrine tumor in multiple endocrine neoplasia type 1 syndrome with intraductal growth into the main pancreatic duct. Pancreas 38:341–342PubMedCrossRefGoogle Scholar
  30. 30.
    Itoh T, Nakayama N, Kikuyama M, Kajiwara T, Yoshizawa A, Manabe T (2009) Endocrine tumor of the pancreas with intraductal spreading. Suizo 24:527–531CrossRefGoogle Scholar
  31. 31.
    Chetty R, El-Shinnawy I (2009) Intraductal pancreatic neuroendocrine tumor. Endocr Pathol 20:262–266PubMedCrossRefGoogle Scholar
  32. 32.
    Kobayashi S, Asakura T, Ohike N, Enomoto T, Sakurai J, Koizumi S, Watanabe T, Nakano H, Otsubo T (2010) Mixed acinar-endocrine carcinoma of the pancreas with intraductal growth into the main pancreatic duct: report of a case. Surg Today 40:380–384PubMedCrossRefGoogle Scholar
  33. 33.
    Maitra A, Krueger JE, Tascilar M, Offerhaus GJ, Angeles-Angeles A, Klimstra DS, Hruban RH, Albores-Saavedra J (2000) Carcinoid tumors of the extrahepatic bile ducts: a study of seven cases. Am J Surg Pathol 24:1501–1510PubMedCrossRefGoogle Scholar
  34. 34.
    Hubert C, Sempoux C, Berquin A, Deprez P, Jamar F, Gigot JF (2005) Bile duct carcinoid tumors: an uncommon disease but with a good prognosis? Hepatogastroenterology 52:1042–1047PubMedGoogle Scholar
  35. 35.
    Modlin IM, Shapiro MD, Kidd M (2005) An analysis of rare carcinoid tumors: clarifying these clinical conundrums. World J Surg 29:92–101PubMedCrossRefGoogle Scholar
  36. 36.
    Nesi G, Lombardi A, Batignani G, Ficari F, Rubio CA, Tonelli F (2006) Well-differentiated endocrine tumor of the distal common bile duct: a case study and literature review. Virchows Arch 449:104–111PubMedCrossRefGoogle Scholar
  37. 37.
    Albores-Saavedra J, Batich K, Hossain S, Henson DE, Schwartz AM (2009) Carcinoid tumors and small-cell carcinomas of the gallbladder and extrahepatic bile ducts: a comparative study based on 221 cases from the surveillance, epidemiology, and end results program. Ann Diagn Pathol 13:378–383PubMedCrossRefGoogle Scholar
  38. 38.
    Carlei F, Barsotti P, Crescenzi A, Bologna M, Muzi P, Schietroma M, Lomanto D, LeZoche E, Simi M, Speranza MS (1993) Neuroendocrine epithelial cells of the main pancreatic ducts and ampulla. Ital J Gastroenterol 25:171–173PubMedGoogle Scholar
  39. 39.
    Maurer CA, Baer HU, Dyong TH, Mueller-Garamvoelgyi E, Friess H, Ruchti C, Reubi JC, Büchler MW (1996) Carcinoid of the pancreas: clinical characteristics and morphological features. Eur J Cancer 32A:1109–1116PubMedCrossRefGoogle Scholar
  40. 40.
    Mao C, el Attar A, Domenico DR, Kim K, Howard JM (1998) Carcinoid tumors of the pancreas. Status report based on two cases and review of the world's literature. Int J Pancreatol 23:153–164PubMedCrossRefGoogle Scholar
  41. 41.
    Soga J (2005) Carcinoids of the pancreas: an analysis of 156 cases. Cancer 104:1180–1187PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  • Thomas Walter
    • 1
    • 2
    • 3
  • Valérie Hervieu
    • 2
    • 3
    • 4
  • Mustapha Adham
    • 1
    • 3
  • Rodica Gincul
    • 1
  • Gilles Poncet
    • 1
    • 2
  • Frank Pilleul
    • 3
    • 5
  • Jean-Alain Chayvialle
    • 1
    • 3
  • Christian Partensky
    • 1
    • 3
  • Catherine Lombard-Bohas
    • 1
  • Jean-Yves Scoazec
    • 2
    • 3
    • 4
    • 6
    Email author
  1. 1.Hospices Civils de Lyon, Hôpital Edouard Herriot, Fédération des Spécialités DigestivesLyonFrance
  2. 2.INSERM, UMR S865, IFR62, Faculté LaennecLyonFrance
  3. 3.Université de Lyon, Université Lyon 1VilleurbanneFrance
  4. 4.Hospices Civils de Lyon, Hôpital Edouard Herriot, Service Central d’Anatomie et Cytologie PathologiquesLyonFrance
  5. 5.Hospices Civils de Lyon, Hôpital Edouard Herriot, Service de Radiologie DigestiveLyonFrance
  6. 6.Anatomie PathologiqueHôpital Edouard HerriotLyonFrance

Personalised recommendations