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Less Common Solid Tumors of Pancreas

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Pancreatic Masses

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Abstract

Primary neoplastic change can occur in all of the histological components of the pancreas. The term “pancreatic cancer” is commonly used to describe pancreatic ductal adenocarcinoma and its associated variants, which arise from the ductal cells and account for 85–90 % of all pancreatic neoplasms. However, neoplasms can also arise from other components of the gland, including stem cells, neuroendocrine cells, lymphoid and stromal cells. Rarely, metastases to the gland also occur. It is important to recognize and understand these tumors as their natural history can differ significantly from pancreatic adenocarcinomas.

After pancreatic adenocarcinoma, pancreatic endocrine tumors are the second most common type of pancreatic tumors and may account for 3 % of pancreatic neoplasms. They were assumed to arise from islet cells, but are now believed to be a group of epithelial neoplasms that are derived from multipotential stem cells of endodermal origin with predominant neuroendocrine differentiation and are classified as pancreatic neuroendocrine tumors (PanNETs). Most pancreatic neuroendocrine tumors are nonfunctional and are increasingly identified at an earlier stage with a greater use of cross-sectional abdominal imaging. Functional PanNETs are neuroendocrine tumors that cause clinical hormonal syndromes and may be recognized by characteristic clinical presentations and serum biomarkers.

There is also a subset of rare pancreatic tumors which includes solid pseudopapillary tumor, acinar cell carcinoma, primary pancreatic lymphoma, and pancreatoblastomas. Some of these are much more indolent and may be differentiated from pancreatic adenocarcinoma and neuroendocrine tumors by histologic and radiographic characteristics. This chapter reviews PanNETs and less common solid tumors of the pancreas with their clinical and imaging features as well as advances in the diagnosis, staging, and management.

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References

  1. Capella C, Heitz PU, Hofler H, Solcia E, Kloppel G. Revised classification of neuroendocrine tumours of the lung, pancreas and gut. Virchows Arch. 1995;425(6):547–60.

    Article  CAS  PubMed  Google Scholar 

  2. Sadaria MR, Hruban RH, Edil BH. Advancements in pancreatic neuroendocrine tumors. Expert Rev Gastroenterol Hepatol. 2013;7(5):477–90.

    Article  CAS  PubMed  Google Scholar 

  3. Fesinmeyer MD, Austin MA, Li CI, De Roos AJ, Bowen DJ. Differences in survival by histologic type of pancreatic cancer. Cancer Epidemiol Biomarkers Prev. 2005;14(7):1766–73.

    Article  PubMed  Google Scholar 

  4. Oberg K. Pancreatic endocrine tumors. Semin Oncol. 2010;37(6):594–618.

    Article  PubMed  CAS  Google Scholar 

  5. Metz DC, Jensen RT. Gastrointestinal neuroendocrine tumors: pancreatic endocrine tumors. Gastroenterology. 2008;135(5):1469–92.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  6. Bosman FT. WHO classification of tumours of the digestive system. 4th ed. Lyon: International Agency for Research on Cancer; 2010. p. 417.

    Google Scholar 

  7. Halfdanarson TR, Rabe KG, Rubin J, Petersen GM. Pancreatic neuroendocrine tumors (PNETs): incidence, prognosis and recent trend toward improved survival. Ann Oncol. 2008;19(10):|1727–33.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  8. Kimura W, Kuroda A, Morioka Y. Clinical pathology of endocrine tumors of the pancreas. Analysis of autopsy cases. Dig Dis Sci. 1991;36(7):933–42.

    Article  CAS  PubMed  Google Scholar 

  9. Fraenkel M, Kim MK, Faggiano A, Valk GD. Epidemiology of gastroenteropancreatic neuroendocrine tumours. Best Pract Res Clin Gastroenterol. 2012;26(6):691–703.

    Article  CAS  PubMed  Google Scholar 

  10. Ito T, Sasano H, Tanaka M, Osamura RY, Sasaki I, Kimura W, et al. Epidemiological study of gastroenteropancreatic neuroendocrine tumors in Japan. J Gastroenterol. 2010;45(2):234–43.

    Article  PubMed  Google Scholar 

  11. Yao JC, Hassan M, Phan A, Dagohoy C, Leary C, Mares JE, et al. One hundred years after “carcinoid”: epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol. 2008;26(18):3063–72.

    Article  PubMed  Google Scholar 

  12. Yao JC, Eisner MP, Leary C, Dagohoy C, Phan A, Rashid A, et al. Population-based study of islet cell carcinoma. Ann Surg Oncol. 2007;14(12):3492–500.

    Article  PubMed Central  PubMed  Google Scholar 

  13. Lawrence B, Gustafsson BI, Chan A, Svejda B, Kidd M, Modlin IM. The epidemiology of gastroenteropancreatic neuroendocrine tumors. Endocrinol Metab Clin North Am. 2011;40(1):1–18. 1.

    Article  PubMed  Google Scholar 

  14. Pieterman CR, Vriens MR, Dreijerink KM, van der Luijt RB, Valk GD. Care for patients with multiple endocrine neoplasia type 1: the current evidence base. Fam Cancer. 2011;10(1):157–71.

    Article  CAS  PubMed  Google Scholar 

  15. de Wilde RF, Edil BH, Hruban RH, Maitra A. Well-differentiated pancreatic neuroendocrine tumors: from genetics to therapy. Nat Rev Gastroenterol Hepatol. 2012;9(4):199–208.

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  16. Vagefi PA, Razo O, Deshpande V, McGrath DJ, Lauwers GY, Thayer SP, et al. Evolving patterns in the detection and outcomes of pancreatic neuroendocrine neoplasms: the Massachusetts General Hospital experience from 1977 to 2005. Arch Surg. 2007;142(4):347–54.

    Article  PubMed Central  PubMed  Google Scholar 

  17. Cheema A, Weber J, Strosberg JR. Incidental detection of pancreatic neuroendocrine tumors: an analysis of incidence and outcomes. Ann Surg Oncol. 2012;19(9):2932–6.

    Article  PubMed  Google Scholar 

  18. Madura JA, Cummings OW, Wiebke EA, Broadie TA, Goulet Jr RL, Howard TJ. Nonfunctioning islet cell tumors of the pancreas: a difficult diagnosis but one worth the effort. Am Surg. 1997;63(7):573–7. discussion 7–8.

    CAS  PubMed  Google Scholar 

  19. Matthews BD, Heniford BT, Reardon PR, Brunicardi FC, Greene FL. Surgical experience with nonfunctioning neuroendocrine tumors of the pancreas. Am Surg. 2000;66(12):1116–22. discussion 22–3.

    CAS  PubMed  Google Scholar 

  20. Service FJ, McMahon MM, O’Brien PC, Ballard DJ. Functioning insulinoma—incidence, recurrence, and long-term survival of patients: a 60-year study. Mayo Clin Proc. 1991;66(7):711–9.

    Article  CAS  PubMed  Google Scholar 

  21. DeVita Jr VT, Lawrence TS, Rosenberg SA, editors. DeVita, Hellman, and Rosenberg’s cancer: principles & practice of oncology. Philadelphia: Lippincott Williams & Wilkins; 2008. Available from: http://catdir.loc.gov/catdir/enhancements/fy0811/2008006079-d.html. http://catdir.loc.gov/catdir/enhancements/fy0811/2008006079-d.html, http://www.lww.com/product/DeVita-Hellman-and-Rosenbergs-Cancer/?978-0-7817-7207-5.

  22. Placzkowski KA, Vella A, Thompson GB, Grant CS, Reading CC, Charboneau JW, et al. Secular trends in the presentation and management of functioning insulinoma at the Mayo Clinic, 1987–2007. J Clin Endocrinol Metab. 2009;94(4):1069–73.

    Article  CAS  PubMed  Google Scholar 

  23. Berna MJ, Hoffmann KM, Serrano J, Gibril F, Jensen RT. Serum gastrin in Zollinger–Ellison syndrome: I. Prospective study of fasting serum gastrin in 309 patients from the National Institutes of Health and comparison with 2229 cases from the literature. Medicine. 2006;85(6):295–330.

    Article  CAS  PubMed  Google Scholar 

  24. Roy PK, Venzon DJ, Shojamanesh H, Abou-Saif A, Peghini P, Doppman JL, et al. Zollinger–Ellison syndrome. Clinical presentation in 261 patients. Medicine. 2000;79(6):379–411.

    Article  CAS  PubMed  Google Scholar 

  25. Norton JA. Neuroendocrine tumors of the pancreas and duodenum. Curr Probl Surg. 1994;31(2):77–156.

    Article  CAS  PubMed  Google Scholar 

  26. Gibril F, Doppman JL, Reynolds JC, Chen CC, Sutliff VE, Yu F, et al. Bone metastases in patients with gastrinomas: a prospective study of bone scanning, somatostatin receptor scanning, and magnetic resonance image in their detection, frequency, location, and effect of their detection on management. J Clin Oncol. 1998;16(3):1040–53.

    CAS  PubMed  Google Scholar 

  27. Norton JA, Alexander HR, Fraker DL, Venzon DJ, Gibril F, Jensen RT. Possible primary lymph node gastrinoma: occurrence, natural history, and predictive factors: a prospective study. Ann Surg. 2003;237(5):650–7. discussion 7–9.

    PubMed Central  PubMed  Google Scholar 

  28. Zollinger RM, Ellison EH. Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas. 1955. CA Cancer J Clin. 1989;39(4):231–47.

    Article  CAS  PubMed  Google Scholar 

  29. Meriney DK. Pathophysiology and management of VIPoma: a case study. Oncol Nurs Forum. 1996;23(6):941–8. quiz 9–50.

    CAS  PubMed  Google Scholar 

  30. Bloom SR, Yiangou Y, Polak JM. Vasoactive intestinal peptide secreting tumors. Pathophysiological and clinical correlations. Ann N Y Acad Sci. 1988;527:518–27.

    Article  CAS  PubMed  Google Scholar 

  31. Verner JV, Morrison AB. Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia. Am J Med. 1958;25(3):374–80.

    Article  CAS  PubMed  Google Scholar 

  32. Perry RR, Vinik AI. Clinical review 72: diagnosis and management of functioning islet cell tumors. J Clin Endocrinol Metab. 1995;80(8):2273–8.

    CAS  PubMed  Google Scholar 

  33. Smith SL, Branton SA, Avino AJ, Martin JK, Klingler PJ, Thompson GB, et al. Vasoactive intestinal polypeptide secreting islet cell tumors: a 15-year experience and review of the literature. Surgery. 1998;124(6):1050–5.

    Article  CAS  PubMed  Google Scholar 

  34. Friesen SR. Update on the diagnosis and treatment of rare neuroendocrine tumors. Surg Clin North Am. 1987;67(2):379–93.

    CAS  PubMed  Google Scholar 

  35. Lecorguille M, Hammel P, Couvelard A, O’Toole D, Ratouis A, Belghiti J, et al. [Jejunal vipoma]. Gastroenterologie clinique et biologique. 2004;28(8–9):797–800.

    Google Scholar 

  36. Wilkinson DS. Necrolytic migratory erythema with carcinoma of the pancreas. Trans St Johns Hosp Dermatol Soc. 1973;59(2):244–50.

    CAS  PubMed  Google Scholar 

  37. Boden G. Glucagonomas and insulinomas. Gastroenterol Clin North Am. 1989;18(4):831–45.

    CAS  PubMed  Google Scholar 

  38. Stacpoole PW. The glucagonoma syndrome: clinical features, diagnosis, and treatment. Endocr Rev. 1981;2(3):347–61.

    Article  CAS  PubMed  Google Scholar 

  39. Pujol RM, Wang CY, el-Azhary RA, Su WP, Gibson LE, Schroeter AL. Necrolytic migratory erythema: clinicopathologic study of 13 cases. Int J Dermatol. 2004;43(1):12–8.

    Article  PubMed  Google Scholar 

  40. Yamada T, Alpers DH. Textbook of gastroenterology. Hoboken: Blackwell; 2009. Available from: http://site.ebrary.com/id/10300962. Materials specified: ebrary http://site.ebrary.com/id/10300962.

    Google Scholar 

  41. Harris GJ, Tio F, Cruz Jr AB. Somatostatinoma: a case report and review of the literature. J Surg Oncol. 1987;36(1):8–16.

    Article  CAS  PubMed  Google Scholar 

  42. Soga J, Yakuwa Y. Somatostatinoma/inhibitory syndrome: a statistical evaluation of 173 reported cases as compared to other pancreatic endocrinomas. J Exp Clin Cancer Res. 1999;18(1):13–22.

    CAS  PubMed  Google Scholar 

  43. Ehehalt F, Saeger HD, Schmidt CM, Grutzmann R. Neuroendocrine tumors of the pancreas. Oncologist. 2009;14(5):456–67.

    Article  CAS  PubMed  Google Scholar 

  44. Filippella M, Davi MV, Doveri G, Lillaz E, Ciccarelli A, Massimetti E, et al. Hyperinsulinemic hypoglycemia associated with ectopic Cushing’s syndrome due to a pancreatic endocrine tumor in a Type 2 diabetes mellitus patient: clinical implications of a rare association. J Endocrinol Invest. 2011;34(3):175–9.

    Article  CAS  PubMed  Google Scholar 

  45. Papazachariou IM, Virlos IT, Williamson RC. Parathyroid hormone-related peptide in pancreatic neuroendocrine tumours associated with hypercalcaemia. HPB (Oxford). 2001;3(3):221–5.

    Article  CAS  Google Scholar 

  46. Wu TJ, Lin CL, Taylor RL, Kvols LK, Kao PC. Increased parathyroid hormone-related peptide in patients with hypercalcemia associated with islet cell carcinoma. Mayo Clin Proc. 1997;72(12):1111–5.

    Article  CAS  PubMed  Google Scholar 

  47. Nauck MA, Meier JJ. Diagnostic accuracy of an “amended” insulin–glucose ratio for the biochemical diagnosis of insulinomas. Ann Intern Med. 2012;157(11):767–75.

    Article  PubMed  Google Scholar 

  48. Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV. The glucagonoma syndrome. Clinical and pathologic features in 21 patients. Medicine. 1996;75(2):53–63.

    Article  CAS  PubMed  Google Scholar 

  49. Ardill JE, O’Dorisio TM. Circulating biomarkers in neuroendocrine tumors of the enteropancreatic tract: application to diagnosis, monitoring disease, and as prognostic indicators. Endocrinol Metab Clin North Am. 2010;39(4):777–90.

    Article  CAS  PubMed  Google Scholar 

  50. Bajetta E, Ferrari L, Martinetti A, Celio L, Procopio G, Artale S, et al. Chromogranin A, neuron specific enolase, carcinoembryonic antigen, and hydroxyindole acetic acid evaluation in patients with neuroendocrine tumors. Cancer. 1999;86(5):858–65.

    Article  CAS  PubMed  Google Scholar 

  51. Friesen SR, Tomita T, Kimmel JR. Pancreatic polypeptide update: its roles in detection of the trait for multiple endocrine adenopathy syndrome, type I and pancreatic polypeptide-secreting tumors. Surgery. 1983;94(6):1028–37.

    CAS  PubMed  Google Scholar 

  52. Nehar D, Lombard-Bohas C, Olivieri S, Claustrat B, Chayvialle JA, Penes MC, et al. Interest of chromogranin A for diagnosis and follow-up of endocrine tumours. Clin Endocrinol (Oxford). 2004;60(5):644–52.

    Article  CAS  Google Scholar 

  53. Kouvaraki MA, Ajani JA, Hoff P, Wolff R, Evans DB, Lozano R, et al. Fluorouracil, doxorubicin, and streptozocin in the treatment of patients with locally advanced and metastatic pancreatic endocrine carcinomas. J Clin Oncol. 2004;22(23):4762–71.

    Article  CAS  PubMed  Google Scholar 

  54. Gouya H, Vignaux O, Augui J, Dousset B, Palazzo L, Louvel A, et al. CT, endoscopic sonography, and a combined protocol for preoperative evaluation of pancreatic insulinomas. Am J Roentgenol. 2003;181(4):987–92.

    Article  Google Scholar 

  55. Rockall AG, Reznek RH. Imaging of neuroendocrine tumours (CT/MR/US). Best Pract Res Clin Endocrinol Metab. 2007;21(1):43–68.

    Article  CAS  PubMed  Google Scholar 

  56. Khashab MA, Yong E, Lennon AM, Shin EJ, Amateau S, Hruban RH, et al. EUS is still superior to multidetector computerized tomography for detection of pancreatic neuroendocrine tumors. Gastrointest Endosc. 2011;73(4):691–6.

    Article  PubMed  Google Scholar 

  57. Ichikawa T, Peterson MS, Federle MP, Baron RL, Haradome H, Kawamori Y, et al. Islet cell tumor of the pancreas: biphasic CT versus MR imaging in tumor detection. Radiology. 2000;216(1):163–71.

    Article  CAS  PubMed  Google Scholar 

  58. Dromain C, de Baere T, Lumbroso J, Caillet H, Laplanche A, Boige V, et al. Detection of liver metastases from endocrine tumors: a prospective comparison of somatostatin receptor scintigraphy, computed tomography, and magnetic resonance imaging. J Clin Oncol. 2005;23(1):70–8.

    Article  PubMed  Google Scholar 

  59. Reidy-Lagunes DL, Gollub MJ, Saltz LB. Addition of octreotide functional imaging to cross-sectional computed tomography or magnetic resonance imaging for the detection of neuroendocrine tumors: added value or an anachronism? J Clin Oncol. 2011;29(3):e74–5.

    Article  PubMed  Google Scholar 

  60. Sundin A. Imaging of neuroendocrine tumors. Expert Opin Med Diagn. 2012;6(5):473–83.

    Article  CAS  PubMed  Google Scholar 

  61. Kim EY. Role of repeated endoscopic ultrasound-guided fine needle aspiration for inconclusive initial cytology result. Clin Endosc. 2013;46(5):540–2.

    Article  PubMed Central  PubMed  Google Scholar 

  62. Abboud B, Boujaoude J. Occult sporadic insulinoma: localization and surgical strategy. World J Gastroenterol. 2008;14(5):657–65.

    Article  PubMed Central  PubMed  Google Scholar 

  63. Jensen RT, Cadiot G, Brandi ML, de Herder WW, Kaltsas G, Komminoth P, et al. ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms: functional pancreatic endocrine tumor syndromes. Neuroendocrinology. 2012;95(2):98–119.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  64. Edge SB, Byrd DR, Compton CC, Fritz AG, Greene FL, Trotti A, editors. AJCC cancer staging handbook. 7th ed. Chicago: American Joint Committee on Cancer; 2010.

    Google Scholar 

  65. Klimstra DS, Modlin IR, Coppola D, Lloyd RV, Suster S. The pathologic classification of neuroendocrine tumors: a review of nomenclature, grading, and staging systems. Pancreas. 2010;39(6):707–12.

    Article  PubMed  Google Scholar 

  66. Ramage JK, Ahmed A, Ardill J, Bax N, Breen DJ, Caplin ME, et al. Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours (NETs). Gut. 2012;61(1):6–32.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  67. Reid MD, Balci S, Saka B, Adsay NV. Neuroendocrine tumors of the pancreas: current concepts and controversies. Endocr Pathol. 2014;25(1):65–79.

    Article  CAS  PubMed  Google Scholar 

  68. Travis WD, Rush W, Flieder DB, Falk R, Fleming MV, Gal AA, et al. Survival analysis of 200 pulmonary neuroendocrine tumors with clarification of criteria for atypical carcinoid and its separation from typical carcinoid. Am J Surg Pathol. 1998;22(8):934–44.

    Article  CAS  PubMed  Google Scholar 

  69. Strosberg JR, Cheema A, Weber JM, Ghayouri M, Han G, Hodul PJ, et al. Relapse-free survival in patients with nonmetastatic, surgically resected pancreatic neuroendocrine tumors: an analysis of the AJCC and ENETS staging classifications. Ann Surg. 2012;256(2):321–5.

    Article  PubMed  Google Scholar 

  70. Strosberg JR, Cheema A, Weber J, Han G, Coppola D, Kvols LK. Prognostic validity of a novel American Joint Committee on Cancer Staging Classification for pancreatic neuroendocrine tumors. J Clin Oncol. 2011;29(22):3044–9.

    Article  PubMed  Google Scholar 

  71. Papavramidis T, Papavramidis S. Solid pseudopapillary tumors of the pancreas: review of 718 patients reported in English literature. J Am Coll Surg. 2005;200(6):965–72.

    Article  PubMed  Google Scholar 

  72. Yu PF, Hu ZH, Wang XB, Guo JM, Cheng XD, Zhang YL, et al. Solid pseudopapillary tumor of the pancreas: a review of 553 cases in Chinese literature. World J Gastroenterol. 2010;16(10):1209–14.

    Article  PubMed Central  PubMed  Google Scholar 

  73. Kato T, Egawa N, Kamisawa T, Tu Y, Sanaka M, Sakaki N, et al. A case of solid pseudopapillary neoplasm of the pancreas and tumor doubling time. Pancreatology. 2002;2(5):495–8.

    Article  PubMed  Google Scholar 

  74. Madan AK, Weldon CB, Long WP, Johnson D, Raafat A. Solid and papillary epithelial neoplasm of the pancreas. J Surg Oncol. 2004;85(4):193–8.

    Article  PubMed  Google Scholar 

  75. Tien YW, Ser KH, Hu RH, Lee CY, Jeng YM, Lee PH. Solid pseudopapillary neoplasms of the pancreas: is there a pathologic basis for the observed gender differences in incidence? Surgery. 2005;137(6):591–6.

    Article  PubMed  Google Scholar 

  76. Abraham SC, Klimstra DS, Wilentz RE, Yeo CJ, Conlon K, Brennan M, et al. Solid-pseudopapillary tumors of the pancreas are genetically distinct from pancreatic ductal adenocarcinomas and almost always harbor beta-catenin mutations. Am J Pathol. 2002;160(4):1361–9.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  77. Maitra A, Weinberg AG, Schneider N, Patterson K. Detection of t(11;22)(q24;q12) translocation and EWS-FLI-1 fusion transcript in a case of solid pseudopapillary tumor of the pancreas. Pediatr Dev Pathol. 2000;3(6):603–5.

    Article  CAS  PubMed  Google Scholar 

  78. Kosmahl M, Seada LS, Janig U, Harms D, Kloppel G. Solid-pseudopapillary tumor of the pancreas: its origin revisited. Virchows Arch. 2000;436(5):473–80.

    Article  CAS  PubMed  Google Scholar 

  79. Klimstra DS. Nonductal neoplasms of the pancreas. Mod Pathol. 2007;20 Suppl 1:S94–112.

    Article  PubMed  Google Scholar 

  80. Nishihara K, Nagoshi M, Tsuneyoshi M, Yamaguchi K, Hayashi I. Papillary cystic tumors of the pancreas. Assessment of their malignant potential. Cancer. 1993;71(1):82–92.

    Article  CAS  PubMed  Google Scholar 

  81. Mortenson MM, Katz MH, Tamm EP, Bhutani MS, Wang H, Evans DB, et al. Current diagnosis and management of unusual pancreatic tumors. Am J Surg. 2008;196(1):100–13.

    Article  PubMed  Google Scholar 

  82. Yao X, Ji Y, Zeng M, Rao S, Yang B. Solid pseudopapillary tumor of the pancreas: cross-sectional imaging and pathologic correlation. Pancreas. 2010;39(4):486–91.

    Article  PubMed  Google Scholar 

  83. Wisnoski NC, Townsend Jr CM, Nealon WH, Freeman JL, Riall TS. 672 patients with acinar cell carcinoma of the pancreas: a population-based comparison to pancreatic adenocarcinoma. Surgery. 2008;144(2):141–8.

    Article  PubMed  Google Scholar 

  84. Holen KD, Klimstra DS, Hummer A, Gonen M, Conlon K, Brennan M, et al. Clinical characteristics and outcomes from an institutional series of acinar cell carcinoma of the pancreas and related tumors. J Clin Oncol. 2002;20(24):4673–8.

    Article  PubMed  Google Scholar 

  85. Kitagami H, Kondo S, Hirano S, Kawakami H, Egawa S, Tanaka M. Acinar cell carcinoma of the pancreas: clinical analysis of 115 patients from Pancreatic Cancer Registry of Japan Pancreas Society. Pancreas. 2007;35(1):42–6.

    Article  PubMed  Google Scholar 

  86. Klimstra DS, Heffess CS, Oertel JE, Rosai J. Acinar cell carcinoma of the pancreas. A clinicopathologic study of 28 cases. Am J Surg Pathol. 1992;16(9):815–37.

    Article  CAS  PubMed  Google Scholar 

  87. Schmidt CM, Matos JM, Bentrem DJ, Talamonti MS, Lillemoe KD, Bilimoria KY. Acinar cell carcinoma of the pancreas in the United States: prognostic factors and comparison to ductal adenocarcinoma. J Gastrointest Surg. 2008;12(12):2078–86.

    Article  PubMed  Google Scholar 

  88. Abraham SC, Wu TT, Hruban RH, Lee JH, Yeo CJ, Conlon K, et al. Genetic and immunohistochemical analysis of pancreatic acinar cell carcinoma: frequent allelic loss on chromosome 11p and alterations in the APC/beta-catenin pathway. Am J Pathol. 2002;160(3):953–62.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  89. Abraham SC, Wu TT, Klimstra DS, Finn LS, Lee JH, Yeo CJ, et al. Distinctive molecular genetic alterations in sporadic and familial adenomatous polyposis-associated pancreatoblastomas: frequent alterations in the APC/beta-catenin pathway and chromosome 11p. Am J Pathol. 2001;159(5):1619–27.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  90. Rigaud G, Moore PS, Zamboni G, Orlandini S, Taruscio D, Paradisi S, et al. Allelotype of pancreatic acinar cell carcinoma. Int J Cancer. 2000;88(5):772–7.

    Article  CAS  PubMed  Google Scholar 

  91. Hoorens A, Lemoine NR, McLellan E, Morohoshi T, Kamisawa T, Heitz PU, et al. Pancreatic acinar cell carcinoma. An analysis of cell lineage markers, p53 expression, and Ki-ras mutation. Am J Pathol. 1993;143(3):685–98.

    PubMed Central  CAS  PubMed  Google Scholar 

  92. Seth AK, Argani P, Campbell KA, Cameron JL, Pawlik TM, Schulick RD, et al. Acinar cell carcinoma of the pancreas: an institutional series of resected patients and review of the current literature. J Gastrointest Surg. 2008;12(6):1061–7.

    Article  PubMed  Google Scholar 

  93. Chiou YY, Chiang JH, Hwang JI, Yen CH, Tsay SH, Chang CY. Acinar cell carcinoma of the pancreas: clinical and computed tomography manifestations. J Comput Assist Tomogr. 2004;28(2):180–6.

    Article  PubMed  Google Scholar 

  94. Tatli S, Mortele KJ, Levy AD, Glickman JN, Ros PR, Banks PA, et al. CT and MRI features of pure acinar cell carcinoma of the pancreas in adults. Am J Roentgenol. 2005;184(2):511–9.

    Article  Google Scholar 

  95. Zucca E, Roggero E, Bertoni F, Cavalli F. Primary extranodal non-Hodgkin’s lymphomas. Part 1: gastrointestinal, cutaneous and genitourinary lymphomas. Ann Oncol. 1997;8(8):727–37.

    Article  CAS  PubMed  Google Scholar 

  96. Freeman C, Berg JW, Cutler SJ. Occurrence and prognosis of extranodal lymphomas. Cancer. 1972;29(1):252–60.

    Article  CAS  PubMed  Google Scholar 

  97. Baylor SM, Berg JW. Cross-classification and survival characteristics of 5,000 cases of cancer of the pancreas. J Surg Oncol. 1973;5(4):335–58.

    Article  CAS  PubMed  Google Scholar 

  98. Nishimura R, Takakuwa T, Hoshida Y, Tsujimoto M, Aozasa K. Primary pancreatic lymphoma: clinicopathological analysis of 19 cases from Japan and review of the literature. Oncology. 2001;60(4):322–9.

    Article  CAS  PubMed  Google Scholar 

  99. Nayer H, Weir EG, Sheth S, Ali SZ. Primary pancreatic lymphomas: a cytopathologic analysis of a rare malignancy. Cancer. 2004;102(5):315–21.

    Article  PubMed  Google Scholar 

  100. Behrns KE, Sarr MG, Strickler JG. Pancreatic lymphoma: is it a surgical disease? Pancreas. 1994;9(5):662–7.

    Article  CAS  PubMed  Google Scholar 

  101. Koniaris LG, Lillemoe KD, Yeo CJ, Abrams RA, Colemann J, Nakeeb A, et al. Is there a role for surgical resection in the treatment of early-stage pancreatic lymphoma? J Am Coll Surg. 2000;190(3):319–30.

    Article  CAS  PubMed  Google Scholar 

  102. Saif MW. Primary pancreatic lymphomas. JOP. 2006;7(3):262–73.

    PubMed  Google Scholar 

  103. Merkle EM, Bender GN, Brambs HJ. Imaging findings in pancreatic lymphoma: differential aspects. Am J Roentgenol. 2000;174(3):671–5.

    Article  CAS  Google Scholar 

  104. Dawson IM, Cornes JS, Morson BC. Primary malignant lymphoid tumours of the intestinal tract. Report of 37 cases with a study of factors influencing prognosis. Br J Surg. 1961;49:80–9.

    Article  CAS  PubMed  Google Scholar 

  105. Salman B, Brat G, Yoon YS, Hruban RH, Singhi AD, Fishman EK, et al. The diagnosis and surgical treatment of pancreatoblastoma in adults: a case series and review of the literature. J Gastrointest Surg. 2013;17(12):2153–61.

    Article  PubMed  Google Scholar 

  106. Bien E, Godzinski J, Dall’igna P, Defachelles AS, Stachowicz-Stencel T, Orbach D, et al. Pancreatoblastoma: a report from the European cooperative study group for paediatric rare tumours (EXPeRT). Eur J Cancer. 2011;47(15):2347–52.

    Article  PubMed  Google Scholar 

  107. Montemarano H, Lonergan GJ, Bulas DI, Selby DM. Pancreatoblastoma: imaging findings in 10 patients and review of the literature. Radiology. 2000;214(2):476–82.

    Article  CAS  PubMed  Google Scholar 

  108. Roland CF, van Heerden JA. Nonpancreatic primary tumors with metastasis to the pancreas. Surg Gynecol Obstet. 1989;168(4):345–7.

    CAS  PubMed  Google Scholar 

  109. Adler H, Redmond CE, Heneghan HM, Swan N, Maguire D, Traynor O, et al. Pancreatectomy for metastatic disease: a systematic review. Eur J Surg Oncol. 2014;40(4):379–86.

    Article  CAS  PubMed  Google Scholar 

  110. Ghavamian R, Klein KA, Stephens DH, Welch TJ, LeRoy AJ, Richardson RL, et al. Renal cell carcinoma metastatic to the pancreas: clinical and radiological features. Mayo Clin Proc. 2000;75(6):581–5.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine, Atlanta, GA, USA

    Saurabh Chawla M.D., Parit Mekaroonkamol M.D. & Field F. Willingham M.D., M.P.H.

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  1. Saurabh Chawla M.D.
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  2. Parit Mekaroonkamol M.D.
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  3. Field F. Willingham M.D., M.P.H.
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Correspondence to Field F. Willingham M.D., M.P.H. .

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Editors and Affiliations

  1. Division of Gastroenterology, University of Florida, Gainesville, Florida, USA

    Mihir S. Wagh

  2. Division of Gastroenterology, University of Florida, Gainesville, Florida, USA

    Peter V. Draganov

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Chawla, S., Mekaroonkamol, P., Willingham, F.F. (2016). Less Common Solid Tumors of Pancreas. In: Wagh, M., Draganov, P. (eds) Pancreatic Masses. Springer, Cham. https://doi.org/10.1007/978-3-319-19677-0_3

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  • DOI: https://doi.org/10.1007/978-3-319-19677-0_3

  • Publisher Name: Springer, Cham

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  • Online ISBN: 978-3-319-19677-0

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