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Pancreatic Acinar Cell Carcinoma: A Multi-institutional Study

  • SSAT Poster Presentation Manuscript
  • Published:
Journal of Gastrointestinal Surgery Aims and scope

Abstract

Introduction

The presentation and outcome of patients with acinar cell carcinoma (ACC) of the pancreas compared to the more common ductal cell adenocarcinoma (DCA) may be distinct. This study combines the experience with ACC from multiple academic institutions to better understand its natural history and outcomes.

Methods

This study is a multi-institutional retrospective review of patients with ACC.

Results

Between 1988 and 2008, 17 patients were identified with pathologically confirmed ACC. Median age at presentation was 59 years. Common presenting symptoms were abdominal pain (60%), back pain (50%), and weight loss (45%). Fifteen patients underwent 16 operations: pancreaticoduodenectomy (nine), distal pancreatectomy (four), and exploratory laparotomy (three). Mean tumor size was 5.3 cm. American Joint Commission on Cancer tumor stages were stage I (two), stage II (eight), stage III (four), and stage IV (three). Overall, 1- and 5-year survival rates were 88% and 50%, respectively. In resected cases (13), 1- and 5-year survival rates were 92% and 53%, respectively. Median survival in resected cases was 61 months. This is in contrast to 1,608 patients with ductal cell adenocarcinoma who underwent resection identified from recent literature reports where the average median survival was only 24 months. There was no discernable difference in the outcomes of patients with ACC between United States and Germany patients.

Conclusion

Acinar cell carcinoma of the pancreas is rare and appears to have a presentation and outcome distinct from the more common pancreatic DCA. Based upon these data, the outcome of patients with ACC is superior to that of DCA.

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References

  1. Williams JA. Regulation of pancreatic acinar cell function. Curr Opin Gastroenterol 2006;22:498–504.

    Article  PubMed  Google Scholar 

  2. Berner. Subkutane fettgewebsnekose. Virchow Arch Pathol Anat 1908;193:510–518.

    Article  Google Scholar 

  3. Robertson JC, Eeles GH. Syndrome associated with pancreatic acinar cell carcinoma. Br Med J 1970;2:708–709.

    Article  CAS  PubMed  Google Scholar 

  4. 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;35:42–46.

    Article  PubMed  Google Scholar 

  5. Mulkeen AL, Yoo PS, Cha C. Less common neoplasms of the pancreas. World J Gastroenterol 2006;12:3180–3185.

    PubMed  Google Scholar 

  6. Chen J, Baithun SI. Morphological study of 391 cases of exocrine pancreatic tumours with special reference to the classification of exocrine pancreatic carcinoma. J Pathol 1985;146:17–29.

    Article  CAS  PubMed  Google Scholar 

  7. Hartman GG, Ni H, Pickleman J. Acinar cell carcinoma of the pancreas. Arch Pathol Lab Med 2001;125:1127–1128.

    CAS  PubMed  Google Scholar 

  8. Ordonez NG. Pancreatic acinar cell carcinoma. Adv Anat Pathol 2001;8:144–159.

    Article  CAS  PubMed  Google Scholar 

  9. Ordonez NG, Mackay B. Acinar cell carcinoma of the pancreas. Ultrastruct Pathol 2000;24:227–241.

    Article  CAS  PubMed  Google Scholar 

  10. 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;16:815–837.

    Article  CAS  PubMed  Google Scholar 

  11. Holen KD, Klimstra DS, Hummer A, Gonen M, Conlon D, Brennan M, Saltz LB. Clinical characteristics and outcomes from an institutional series of acinar cell carcinoma of the pancreas and related tumors. J Clin Oncol 2002;20:4673–4678.

    Article  PubMed  Google Scholar 

  12. Webb JN. Acinar cell neoplasms of the exocrine pancreas. J Clin Pathol 1977;30:103–112.

    Article  CAS  PubMed  Google Scholar 

  13. Cubilla AL, Fitzgerald PJ. Classification of pancreatic cancer (nonendocrine). Mayo Clin Proc 1979;54:449–458.

    CAS  PubMed  Google Scholar 

  14. Khalili M, Wax BN, Reed WP, Schuss A, Drexler S, Weston SR, Katz DS. Radiology–pathology conference. Acinar cell carcinoma of the pancreas. Clin Imaging 2006;30:343–346.

    Article  PubMed  Google Scholar 

  15. Seth AK, Argani P, Campbell KA, Cameron JL, Pawlik TM, Schulick RD, Choti MA, Wolfgang CL. Acinar cell carcinoma of the pancreas: an institutional series of resected patients and review of the current literature. J Gastrointest Surg 2008;12:1061–1067.

    Article  PubMed  Google Scholar 

  16. Nitecki SS, Sarr MG, Colby TV, van Heerden JA. Long-term survival after resection for ductal adenocarcinoma of the pancreas. Is it really improving? Ann Surg 1995;221:59–66.

    Article  CAS  PubMed  Google Scholar 

  17. Sohn TA, Yeo CJ, Cameron JL, Koniaris L, Kaushal S, Abrams RA, Sauter PK, Coleman J, Hruban RH, Lillemoe KD. Resected adenocarcinoma of the pancreas—616 patients: results, outcomes, and prognostic indicators. J Gastrointest Surg 2000;4:567–579.

    Article  CAS  PubMed  Google Scholar 

  18. Breslin TM, Hess KR, Harbison DB, Jean ME, Cleary KR, Dackiw AP, Wolff RA, Abbruzzese JL, Janjan NA, Crane CH, Vauthey JN, Lee JE, Pisters PW, Evans DB. Neoadjuvant chemoradiotherapy for adenocarcinoma of the pancreas: treatment variables and survival duration. Ann Surg Oncol 2001;8:123–132.

    Article  CAS  PubMed  Google Scholar 

  19. Oettle H, Post S, Neuhaus P, Gellert K, Langrehr J, Ridwelski K, Schramm H, Fahlke J, Zuelke C, Burkart C, Gutberlet K, Kettner E, Schmalenberg H, Weigang-Koehler K, Bechstein WO, Niedergethmann M, Schmidt-Wolf I, Roll L, Doerken B, Riess H. Adjuvant chemotherapy with gemcitabine vs observation in patients undergoing curative-intent resection of pancreatic cancer: a randomized controlled trial. JAMA 2007;297:267–277.

    Article  CAS  PubMed  Google Scholar 

  20. Evans DB, Varadhachary GR, Crane CH, Sun CC, Lee JE, Pisters PW, Vauthey JN, Wang H, Cleary KR, Staerkel GA, Charnsangavej C, Lano EA, Ho L, Lenzi R, Abbruzzeese JL, Wolff RA. Preoperative gemcitabine-based chemoradiation for patients with resectable adenocarcinoma of the pancreatic head. J Clin Oncol 2008;26:3496–3502.

    Article  CAS  PubMed  Google Scholar 

  21. Varadhachary GR, Wolff RA, Crane CH, Sun CC, Lee JE, Pisters PW, Vauthey JN, Abdalla E, Wang H, Staerkel GA, Lee JH, Ross WA, Tamm EP, Bhosale PR, Drishnan S, Das P, Ho L, Xiong H, Abbruzzese JL, Evans DB. Preoperative gemcitabine and cisplatin followed by gemcitabine-based chemoradiation for resectable adenocarcinoma of the pancreatic head. J Clin Oncol 2008;26:3487–3495.

    Article  CAS  PubMed  Google Scholar 

  22. Turrini O, Viret F, Moureau-Zabotto L. Ten years experience with neoadjuvant radiochemotherapy for patients with resectable adenocarcinoma of the pancreatic head. Oncology 2009 (in press).

  23. Katz MH, Wang H, Fleming JB, Sun CC, Hwang RF, Wolff RA, Varadhachary G, Abbruzzese JL, Crane CH, Krishnan S, Vauthey JN, Abdalla EK, Lee JE, Pisters PW, Evans DB. Long-term survival after multidisciplinary management of resected pancreatic adenocarcinoma. Ann Surg Oncol 2009;15:836–847.

    Article  Google Scholar 

  24. Wisnoski NC, Townsend CM Jr., Nealon WH, Freeman JL, Riall TS. 672 patients with acinar cell carcinoma of the pancreas: a population-based comparison to pancreatic adenocarcinoma. Surgery 2008;144:141–148.

    Article  PubMed  Google Scholar 

  25. 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;12:2078–2086.

    Article  PubMed  Google Scholar 

  26. Ohike N, Kosmahl M, Kloppel G. Mixed acinar–endocrine carcinoma of the pancreas. A clinicopathological study and comparison with acinar–cell carcinoma. Virchows Arch 2004;445:231–235.

    Article  PubMed  Google Scholar 

  27. Morohoshi T, Kanda M, Horie A, Chott A, Dreyer T, Kloppel G, Heitz PU. Immunocytochemical markers of uncommon pancreatic tumors. Acinar cell carcinoma, pancreatoblastoma, and solid cystic (papillary-cystic) tumor. Cancer 1987;59:739–747.

    Article  CAS  PubMed  Google Scholar 

  28. Klimstra DS, Rosai J, Heffess CS. Mixed acinar–endocrine carcinomas of the pancreas. Am J Surg Pathol 1994;18:765–778.

    CAS  PubMed  Google Scholar 

  29. Basturk O, Zamboni G, Klimstra DS, Capelli P, Andea A, Kamel NS, Adsay NV. Intraductal and papillary variants of acinar cell carcinomas: a new addition to the challenging differential diagnosis of intraductal neoplasms. Am J Surg Pathol 2007;31:363–370.

    Article  PubMed  Google Scholar 

  30. Hashimoto M, Matsuda M, Watanabe G, Mori M, Motoi N, Nagai K, Ishibashi M. Acinar cell carcinoma of the pancreas with intraductal growth: report of a case. Pancreas 2003;26:306–308.

    Article  PubMed  Google Scholar 

  31. Svrcek M, Lesurtel M, Lewin M, Afchain P, Fabre M, Scoazec JY, Parc R, Flejou JF. Acinar cell carcinoma of the pancreas with predominant intraductal growth: report of a case. Gastroenterol Clin Biol 2007;31:543–546.

    Article  PubMed  Google Scholar 

  32. Fabre A, Sauvanet A, Flejou JF, Belghiti J, Palazzo L, Ruszniewski P, Degott C, Terris B. Intraductal acinar cell carcinoma of the pancreas. Virchows Arch 2001;438:312–315.

    Article  CAS  PubMed  Google Scholar 

  33. Sawey ET, Johnson JA, Crawford HC. Matrix metalloproteinase 7 controls pancreatic acinar cell transdifferentiation by activating the Notch signaling pathway. Proc Natl Acad Sci USA 2007;104:19327–19332.

    Article  CAS  PubMed  Google Scholar 

  34. Means AL, Meszoely IM, Suzuki K, Miyamoto Y, Rustgi AK, Coffey RJ, Wright CV, Stoffers DA, Leach SD. Pancreatic epithelial plasticity mediated by acinar cell transdifferentiation and generation of nestin-positive intermediates. Development 2005;132:3767–3776.

    Article  CAS  PubMed  Google Scholar 

  35. Schmid RM. Acinar-to-ductal metaplasia in pancreatic cancer development. J Clin Invest 2002;109:1403–1404.

    CAS  PubMed  Google Scholar 

  36. Zhu L, Tran T, Rukstalis JM, Sun P, Damsz B, Konieczny SF. Inhibition of Mist1 homodimer formation induces pancreatic acinar-to-ductal metaplasia. Mol Cell Biol 2004;24:2673–2681.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Surgery, Indiana University School of Medicine, Indianapolis, IN, USA

    Jesus M. Matos, C. Max Schmidt, Olivier Turrini & Keith D. Lillemoe

  2. Department of Pathology, Indiana University School of Medicine, Indianapolis, IN, USA

    Narasimhan P. Agaram

  3. Department of Surgery, University Hospital Mannheim, Mannheim, Germany

    Marco Niedergethmann

  4. Department of Surgery, University Hospital Dresden, Dresden, Germany

    Hans Detlev Saeger & Robert Grützmann

  5. Department of Surgery, Vanderbilt University Medical Center, Nashville, TN, USA

    Nipun Merchant

  6. Division of Biostatistics, Indiana University School of Medicine, Indianapolis, IN, USA

    Cynthia S. Johnson

  7. Department of Biochemistry & Molecular Biology, Indiana University School of Medicine, 1044 W Walnut St R4-039, Indianapolis, IN, 46202, USA

    C. Max Schmidt

Authors
  1. Jesus M. Matos
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  2. C. Max Schmidt
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  3. Olivier Turrini
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  6. Hans Detlev Saeger
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  8. Cynthia S. Johnson
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  9. Keith D. Lillemoe
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Corresponding author

Correspondence to C. Max Schmidt.

Additional information

Jesus M. Matos and C. Max Schmidt contributed equally to this work.

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Matos, J.M., Schmidt, C.M., Turrini, O. et al. Pancreatic Acinar Cell Carcinoma: A Multi-institutional Study. J Gastrointest Surg 13, 1495–1502 (2009). https://doi.org/10.1007/s11605-009-0938-z

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  • DOI: https://doi.org/10.1007/s11605-009-0938-z

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