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The Diagnosis and Surgical Treatment of Pancreatoblastoma in Adults: A Case Series and Review of the Literature

  • Case Report
  • Published:
Journal of Gastrointestinal Surgery

Abstract

Introduction

Pancreatoblastoma is an extremely rare pancreatic neoplasm in adults. The aim of this study is to report our experience with adult pancreatoblastoma as well as review the cases reported in the literature in order to provide guidelines for the management of patients with this rare neoplasm.

Methods

We have encountered three cases of pancreatoblastoma in adults at our institution in addition to the 30 cases reported to date in literature.

Results

The median age of pancreatoblastoma in adults is 37 years (range, 18–78 years); men and women are similarly affected (male/female = 16/17). The behavior of pancreatoblastoma is clearly that of a malignant neoplasm, with local invasion, recurrence, and metastasis. Among the adult reported cases, at diagnosis or operation, metastasis and/or local invasion was found in 14 of 31 adult patients (46 %) (2 patients had no data) The survival was significantly higher in patients with resected tumor (resection only and resection + adjuvant chemo/radiotherapy) when compared to unresected patients (palliative chemo/radiotherapy and no treatment), (p = 0.008, HR = 0.20).

Conclusion

When disease is localized, the treatment of choice is a complete surgical resection. The role of adjuvant chemotherapy or radiotherapy is still unclear based on the very small number of patients treated.

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References

  1. Becker WF. Pancreatoduodectomy for carcinoma of the pancreas in an infant: report of a case. Ann Surg 1957; 145: 864–872.

    Article  PubMed  CAS  Google Scholar 

  2. Horie A, Yano Y, Kotoo Y, Miwa A. Morphogenesis of pancreatoblastoma, infantile carcinoma of the pancreas: report of two cases. Cancer 1977; 39: 247–254.

    Article  PubMed  CAS  Google Scholar 

  3. Palosaari D, Clayton F, Seaman J. Pancreatoblastoma in an adult. Arch Pathol Lab Med. 1986; 110: 650–652.

    PubMed  CAS  Google Scholar 

  4. Klimstra DS, Wenig BM, Adair CF, Heffess CS. Pancreatoblastoma. A clinicopathologic study and review of the literature. Am J Surg Pathol. 1995; 19: 1371–1389.

    Article  CAS  Google Scholar 

  5. Comper F, Antonello D, Beghelli S, Gobbo S, Montagna L, Pederzoli P, Chilosi M, Scarpa A. Expression pattern of claudins 5 and 7 distinguishes solid-pseudo papillary from pancreatoblastoma, acinar cell and endocrine tumors of the pancreas. Am J Surg Pathol. 2009; 33: 768–774.

    Article  PubMed  Google Scholar 

  6. Ohike N, Yamochi T, Shiokawa A, Yoshida T, Yamazaki T, Date Y, Morohoshi T.A peculiar variant of pancreatoblastoma in an adult. Pancreas. 2008; 36: 320–322.

    Google Scholar 

  7. Chung EM, Travis MD, Conran RM. Pancreatic tumors in children: radiologic-pathologic correlation. Radiographics 2006; 26: 1211–1238.

    Article  PubMed  Google Scholar 

  8. Saif MW. Pancreatoblastoma. J Pancreas (Online) 2007; 8: 55–63.

    Google Scholar 

  9. Pitman MB, Faquin WC. The fine-needle aspiration biopsy cytology of pancreatoblastoma. Diagn Cytopathol. 2004; 31: 402–406.

    Article  PubMed  Google Scholar 

  10. Sheng L, Weixia Z, Longhai Y, Jinming Y. Clinical and biologic analysis of pancreatoblastoma. Pancreas. 2005; 30: 87–90.

    Article  PubMed  Google Scholar 

  11. Du E, Katz M, Weidner N, Yoder S, Moossa AR, Shabaik A. Ampullary pancreatoblastoma in an elderly patient: a case report and review of the literature. Arch Pathol Lab Med. 2003; 127: 1501–1505.

    PubMed  Google Scholar 

  12. Cavallini A, Falconi M, Bortesi L, Crippa S, Barugola G, Butturini G. Pancreatoblastoma in adults: a review of the literature. Pancreatology. 2009; 9: 73–80.

    Article  PubMed  Google Scholar 

  13. Hoorens A, Gebhard F, Kraft K, Lemoine NR, Klöppel G. Pancreatoblastoma in an adult: its separation from acinar cell carcinoma. Virchows Arch. 1994; 424: 485–490.

    Article  PubMed  CAS  Google Scholar 

  14. Dunn JL, Longnecker DS. Pancreatoblastoma in an older adult. Arch Pathol Lab Med. 1995; 119: 547–551.

    PubMed  CAS  Google Scholar 

  15. Levey JM, Banner BF. Adult pancreatoblastoma: a case report and review of the literature. Am J Gastroenterol. 1996; 91: 1841–1844.

    PubMed  CAS  Google Scholar 

  16. Robin E, Terris B, Valverde A, Molas G, Belghiti J, Bernades P, Ruszniewski P. Pancreatoblastoma in adults. Gastroenterol Clin Biol. 1997; 21: 880–883.

    PubMed  CAS  Google Scholar 

  17. Hayasaki N, Miyake N, Takahashi H, Nakamura E, Yamagishi S, Kuno Y, Mori N, Shinoda M, Kimura M, Suzuki T, Tashiro K. A case of pancreatoblastoma in an adult. Nihon Shokakibyo Gakkai Zasshi. 1999; 96: 558–563.

    PubMed  CAS  Google Scholar 

  18. Montemarano H, Lonergan GJ, Bulas DI, Selby DM. Pancreatoblastoma: imaging findings in 10 patients and review of the literature. Radiology. 2000; 214: 476–482.

    Article  PubMed  CAS  Google Scholar 

  19. Mumme T, Büttner R, Peiper C, Schumpelick V. Pancreatoblastoma: a rare malignant neoplasm in early adulthood. Chirurg. 2001; 72: 806–811.

    Article  PubMed  CAS  Google Scholar 

  20. Benoist S, Penna C, Julié C, Malafosse R, Rougier P, Nordlinger B. Prolonged survival after resection of pancreatoblastoma and synchronous liver metastases in an adult. Hepatogastroenterology. 2001; 48: 1340–1342.

    PubMed  CAS  Google Scholar 

  21. Abraham SC, Wu TT, Klimstra DS, Finn LS, Lee JH, Yeo CJ, Cameron JL, Hruban RH. 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: 1619–1627

    Article  PubMed  CAS  Google Scholar 

  22. Gruppioni F, Casadei R, Fusco F, Calculli L, Marrano D, Gavelli G. Adult pancreatoblastoma. A case report. Radiol Med. 2002; b103 (1–2): b119-22

    Google Scholar 

  23. Rosebrook JL, Glickman JN, Mortele KJ. Pancreatoblastoma in an adult woman: sonography, CT, and dynamic gadolinium-enhanced MRI features. Am J Roentgenol. 2005 184 (3 Suppl): S78-81

    Article  Google Scholar 

  24. Zhu LC, Sidhu GS, Cassai ND, Yang GC. Fine-needle aspiration cytology of pancreatoblastoma in a young woman: report of a case and review of the literature. Diagn Cytopathol. 2005; 33: 258–262

    Article  PubMed  Google Scholar 

  25. Rajpal S, Warren RS, Alexander M, Yeh BM, Grenert JP, Hintzen S, Ljung BM, Bergsland EK. Pancreatoblastoma in an adult: case report and review of the literature. J Gastrointest Surg. 2006; 10: 829–836

    Article  PubMed  Google Scholar 

  26. Charlton-Ouw KM, Kaiser CL, Tong GX, Allendorf JD, Chabot JA. Revisiting metastatic adult pancreatoblastoma. A case and review of the literature. JOP. 2008; 9: 733–738

    Google Scholar 

  27. Savastano S, d'Amore ES, Zuccarotto D, Banzato O, Beghetto M, Famengo B. Pancreatoblastoma in an adult patient. A case report. JOP. 2009; 10(2): 192–195

    PubMed  Google Scholar 

  28. Boix E, Yuste A, Meana A, Alcaraz E, Payá A, Arnold C, Picó A, Lluis F. Corticotrophin-releasing hormone-secreting pancreatoblastoma in an adult patient. Pancreas. 2010; 39: 938–939

    Article  PubMed  Google Scholar 

  29. Balasundaram C, Luthra M, Chavalidthamrong D, Chow J, Khan H, Endres P JH. Pancreatoblastoma: A Rare Tumor Still Evolving in Clinical Presentation and Histology. JOP. 2012: 3; 301–303

  30. Gupta AK, Mitra DK, Berry M, Dinda AK, Bhatnagar V. Sonography and CT of pancreatoblastoma in children. Am J Roentgenol. 2000; 174: 1639–1641

    Article  CAS  Google Scholar 

  31. Klimstra DS, Longnecker DS. Pancreablastoma. In: Hamilton SR, Aaltonen LA, eds. WHO Classification of Tumors: Pathology and Genetics of Tumors of the Digestive System. Lyon, France: IARC Press, 2000, pp 244–245

    Google Scholar 

Download references

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Authors and Affiliations

  1. Department of Surgery, The Sol Goldman Pancreatic Cancer Research Center, Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins Medical Institutions, Baltimore, MD, USA

    Bulent Salman, Gabriel Brat, Yoo-Seok Yoon & Christopher L. Wolfgang

  2. Department of Pathology, The Sol Goldman Pancreatic Cancer Research Center, Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins Medical Institutions, Baltimore, MD, USA

    Ralph H. Hruban & Christopher L. Wolfgang

  3. Department of Radiology, The Sol Goldman Pancreatic Cancer Research Center, Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins Medical Institutions, Baltimore, MD, USA

    Elliot K. Fishman

  4. Department of Oncology, The Sol Goldman Pancreatic Cancer Research Center, Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins Medical Institutions, Baltimore, MD, USA

    Ralph H. Hruban, Elliot K. Fishman, Joseph M. Herman & Christopher L. Wolfgang

  5. Department of Radiation Oncology, The Sol Goldman Pancreatic Cancer Research Center, Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins Medical Institutions, Baltimore, MD, USA

    Joseph M. Herman

  6. Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA

    Aatur D. Singhi

  7. Surgery, Pathology and Oncology, Cameron Division of Surgical Oncology, Johns Hopkins Hospital, 685 Blalock, 600 N. Wolfe Street, Baltimore, MD, 21287, USA

    Christopher L. Wolfgang

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  1. Bulent Salman
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Correspondence to Christopher L. Wolfgang.

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Salman, B., Brat, G., Yoon, YS. et al. The Diagnosis and Surgical Treatment of Pancreatoblastoma in Adults: A Case Series and Review of the Literature. J Gastrointest Surg 17, 2153–2161 (2013). https://doi.org/10.1007/s11605-013-2294-2

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  • DOI: https://doi.org/10.1007/s11605-013-2294-2

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