Skip to main content

Advertisement

Log in

Hepatocellular carcinoma and solid pseudopapillary neoplasm of the pancreas complicating familial adenomatous polyposis: two cases and review of the literature

  • Short Communication
  • Published:
Familial Cancer Aims and scope Submit manuscript

Abstract

Familial adenomatous polyposis (FAP) is characterized by colorectal polyposis and extracolonic tumors. Adenocarcinoma of the pancreas and hepatocellular carcinoma are rare in FAP. In this case series, we describe a mother and daughter with FAP who developed a hepatocellular carcinoma and solid pseudopapillary neoplasm of the pancreas, respectively.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2

References

  1. Half E, Bercovich D, Rozen P (2009) Familial adenomatous polyposis. Orphanet J Rare Dis 4(1):1–23

    Article  Google Scholar 

  2. Anaya DA, Chang GJ, Rodriguez-Bigas MA (2008) Extracolonic manifestations of hereditary colorectal cancer syndromes. Clin Colon Rectal Surg 21(04):263–272

    Article  Google Scholar 

  3. McGlynn KA, Petrick JL, El-Serag HB (2021) Epidemiology of hepatocellular carcinoma. Hepatology 73:4–13

    Article  CAS  Google Scholar 

  4. Herbst DA, Reddy KR (2012) Risk factors for hepatocellular carcinoma. Clin Liver Dis 1(6):180–182

    Article  Google Scholar 

  5. Syngal S, Brand RE, Church JM, Giardiello FM, Hampel HL, Burt RW (2015) ACG clinical guideline: genetic testing and management of hereditary gastrointestinal cancer syndromes. Am J Gastroenterol 110(2):223

    Article  Google Scholar 

  6. Veale A (1965) Intestinal polyposis. Eugenics Lab. Memoirs, Series 40. Cambridge University Press, London

  7. Zeze F, Ohsato K, Mitani H, Ohkuma R, Koide O (1983) Hepatocellular carcinoma associated with familial polyposis of the colon. Dis Colon Rectum 26(7):465–468

    Article  CAS  Google Scholar 

  8. Laferla G, Kaye S, Crean G (1988) Hepatocellular and gastric carcinoma associated with familial polyposis coli. J Surg Oncol 38(1):19–21

    Article  CAS  Google Scholar 

  9. Van Steenbergen W, Fevery J, De Groote J, Baert A, Desmet V, Van Eyken P (1989) Hepatocellular carcinoma in a case of familial polyposis coli. Am J Gastroenterol 84(9):1120–1121

    Google Scholar 

  10. Spigelman A, Farmer K, James M, Richman P, Phillips R (1991) Tumours of the liver, bile ducts, pancreas and duodenum in a single patient with familial adenomatous polyposis. J Br Surg 78(8):979–980

    Article  CAS  Google Scholar 

  11. Gruner BA, DeNapoli TS, Andrews W, Tomlinson G, Bowman L, Weitman SD (1998) Hepatocellular carcinoma in children associated with gardner syndrome or familial adenomatous polyposis. J Pediatr Hematol Oncol 20(3):274–278

    Article  CAS  Google Scholar 

  12. Paulson S, Patel C, Patel H (2016) From the gut to the liver: another organ to watch in FAP patients. Case reports in pathology 2016

  13. Law JK, Ahmed A, Singh VK et al (2014) A systematic review of solid-pseudopapillary neoplasms: are these rare lesions? Pancreas 43(3):331

    Article  Google Scholar 

  14. Papavramidis T, Papavramidis S (2005) Solid pseudopapillary tumors of the pancreas: review of 718 patients reported in English literature. J Am Coll Surg 200(6):965–972

    Article  Google Scholar 

  15. Song H, Dong M, Zhou J, Sheng W, Zhong B, Gao W (2017) Solid pseudopapillary neoplasm of the pancreas: clinicopathologic feature, risk factors of malignancy, and survival analysis of 53 cases from a single center. BioMed Res Int 2017:1–7

    Article  Google Scholar 

  16. Mao C, Guvendi M, Domenico DR, Kim K, Thomford NR, Howard JM (1995) Papillary cystic and solid tumors of the pancreas: a pancreatic embryonic tumor? Studies of three cases and cumulative review of the world’s literature. Surgery 118(5):821–828

    Article  CAS  Google Scholar 

  17. Groen EJ, Roos A, Muntinghe FL et al (2008) Extra-intestinal manifestations of familial adenomatous polyposis. Ann Surg Oncol 15(9):2439–2450

    Article  Google Scholar 

  18. Ruo L, Coit DG, Brennan MF, Guillem JG (2002) Long-term follow-up of patients with familial adenomatous polyposis undergoing pancreaticoduodenal surgery. J Gastrointest Surg 6(5):671–675

    Article  Google Scholar 

  19. Farahmand F, Shoaran M, Fariborzi M, Ashjaei B, Monajemzadeh M, Mehdizadeh M (2012) Pancreatic pseudopapillary tumor in association with colonic polyposis. J Med Med Sci 3:447–451

    Google Scholar 

  20. Inoue T, Nishi Y, Okumura F et al (2015) Solid pseudopapillary neoplasm of the pancreas associated with familial adenomatous polyposis. Intern Med 54(11):1349–1355

    Article  Google Scholar 

  21. Naoi D, Koinuma K, Sasanuma H et al (2021) Solid-pseudopapillary neoplasm of the pancreas in a patient with familial adenomatous polyposis: a case report. Surg Case Rep 7(1):1–6

    Article  Google Scholar 

  22. Miller J (2001) Protein family review the wnts. Gene 1:15

    Google Scholar 

  23. Terris B, Pineau P, Bregeaud L et al (1999) Close correlation between β-catenin gene alterations and nuclear accumulation of the protein in human hepatocellular carcinomas. Oncogene 18(47):6583–6588

    Article  CAS  Google Scholar 

  24. de La Coste A, Romagnolo B, Billuart P et al (1998) Somatic mutations of the β-catenin gene are frequent in mouse and human hepatocellular carcinomas. Proc Natl Acad Sci 95(15):8847–8851

    Article  Google Scholar 

Download references

Funding

This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Author information

Authors and Affiliations

Authors

Contributions

CAB was involved in the conception and design of the work, critical revision of the article, and final approval of the version to be published. JEH was involved in data collection, drafting the article, critical revision of the article, and final approval of the version to be published. LL was involved in data collection, critical revision of the article, and final approval of the version to be published. RMW was involved in critical revision of the article, and final approval of the version to be published. CHDK was involved in critical revision of the article, and final approval of the version to be published. KVNM was involved in critical revision of the article, and final approval of the version to be published. DL was involved in critical revision of the article, and final approval of the version to be published.

Corresponding author

Correspondence to Carol A. Burke.

Ethics declarations

Conflict of interest

None of the authors disclose any competing interests.

Ehical approval

Both patients were participants in the IRB approved David G. Jagelman Inherited Colorectal Cancer Registries at the Cleveland Clinic. Reference ID 2884.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

El Halabi, J., LaGuardia, L., Walsh, R.M. et al. Hepatocellular carcinoma and solid pseudopapillary neoplasm of the pancreas complicating familial adenomatous polyposis: two cases and review of the literature. Familial Cancer 22, 77–82 (2023). https://doi.org/10.1007/s10689-022-00305-0

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10689-022-00305-0

Keywords

Navigation