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Juvenile polyposis of the stomach—a novel cause of hypergastrinemia

Nature Reviews Gastroenterology & Hepatology volume 7, pages 583–588 (2010)Cite this article

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Abstract

Background. A 38-year-old female presented with a 3-year history of postprandial abdominal pain, refractory nausea, vomiting and hematemesis. She appeared malnourished and her symptoms were refractory to previous treatment with acid-suppressive drugs, prokinetics and antiemetics. Her medical history was significant for a diagnosis of juvenile polyposis syndrome at the age of 14 resulting in a transverse colectomy, and a diagnosis of Crohn's disease in her residual colon at the age of 35 resulting in a total colectomy.

Investigations. Physical examination, blood analysis, esophagogastroduodenoscopy with biopsy, abdominal endoscopic ultrasound, abdominal CT scan, MRI, 24 h urine analysis, MIBG scintigraphy, ocreotide scintigraphy, fluorodeoxyglucose-PET scan and genetic testing for defined polyposis syndromes (SMAD4, BMPR1A).

Diagnosis. Juvenile polyposis syndrome with outlet obstruction of the stomach and excessive hypergastrinemia.

Management. Continuous acid-suppressive therapy, prokinetic therapy and total parenteral nutrition. Repetitive endoscopic polypectomy (also known as debulking) was performed twice and was followed by gastrectomy with duodenoesophageal anastomosis.

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Figure 1: Extensive gastric polyposis in the case patient.
Figure 2: Hematoxylin and eosin stained section from the case patient's gastrectomy specimen.
Figure 3: Immunohistochemistry findings in the case patient.
Figure 4: Illustration of the differences in cell migration pattern in healthy pyloric mucosa (left) and the polypoid, hyperplastic mucosa (right).

References

  1. Fossmark, R. et al. Spontaneous enterochromaffin-like cell carcinomas in cotton rats (Sigmodon hispidus) are prevented by a somatostatin analogue. Endocr. Relat. Cancer 11, 149–160 (2004).

    Article  CAS  Google Scholar 

  2. Hangen, D., Maltz, G. S., Anderson, J. E. & Knauer, C. M. Marked hypergastrinemia in gastric outlet obstruction. J. Clin. Gastroenterol. 11, 442–444 (1989).

    Article  CAS  Google Scholar 

  3. M'Koma, A. E., Lindquist, K. & Liljeqvist, L. Effect of restorative proctocolectomy on gastric acid secretion and serum gastrin levels: a prospective study. Dis. Colon Rectum 42, 398–402 (1999).

    Article  CAS  Google Scholar 

  4. Dockray, G. J. Clinical endocrinology and metabolism. Gastrin. Best Pract. Res. Clin. Endocrinol. Metab. 18, 555–568 (2004).

    Article  CAS  Google Scholar 

  5. Jorgensen, N. R., Rehfeld, J. F., Bardram, L. & Hilsted, L. Processing-independent analysis in the diagnosis of gastrinomas. Scand. J. Gastroenterol. 33, 379–385 (1998).

    Article  CAS  Google Scholar 

  6. Sandvik, A. K., Brenna, E. & Waldum, H. L. Review article: the pharmacological inhibition of gastric acid secretion--tolerance and rebound. Aliment. Pharmacol. Ther. 11, 1013–1018 (1997).

    Article  CAS  Google Scholar 

  7. Suarez-Pinzon, W. L. et al. Combination therapy with epidermal growth factor and gastrin increases beta-cell mass and reverses hyperglycemia in diabetic NOD mice. Diabetes 54, 2596–2601 (2005).

    Article  CAS  Google Scholar 

  8. Wang, R. N., Rehfeld, J. F., Nielsen, F. C. & Kloppel, G. Expression of gastrin and transforming growth factor-alpha during duct to islet cell differentiation in the pancreas of duct-ligated adult rats. Diabetologia 40, 887–893 (1997).

    Article  CAS  Google Scholar 

  9. Laine, L., Takeuchi, K. & Tarnawski, A. Gastric mucosal defense and cytoprotection: bench to bedside. Gastroenterology 135, 41–60 (2008).

    Article  CAS  Google Scholar 

  10. Friedl, W. et al. Juvenile polyposis: massive gastric polyposis is more common in MADH4 mutation carriers than in BMPR1A mutation carriers. Hum. Genet. 111, 108–111 (2002).

    Article  CAS  Google Scholar 

  11. Zbuk, K. M. & Eng, C. Hamartomatous polyposis syndromes. Nat. Clin. Pract. Gastroenterol. Hepatol. 4, 492–502 (2007).

    Article  CAS  Google Scholar 

  12. Jass, J. R., Williams, C. B., Bussey, H. J. & Morson, B. C. Juvenile polyposis--a precancerous condition. Histopathology 13, 619–630 (1988).

    Article  CAS  Google Scholar 

  13. Bonilla Palacios, J. J. et al. Serum gastrin, pepsinogens, parietal cell and Helicobacter pylori antibodies in patients with gastric polyps. Acta Gastroenterol. Latinoam. 24, 77–82 (1994).

    CAS  PubMed  Google Scholar 

  14. Niv, Y. et al. Hyperplastic gastric polyposis, hypergastrinaemia and colorectal neoplasia: a description of four cases. Eur. J. Gastroenterol. Hepatol. 15, 1361–1366 (2003).

    Article  Google Scholar 

  15. Goswami, R. S. et al. Hyperplastic polyposis and cancer of the colon with gastrinoma of the duodenum. Nat. Clin. Pract. Oncol. 3, 281–284 (2006).

    Article  Google Scholar 

  16. Howe, J. R. et al. The prevalence of MADH4 and BMPR1A mutations in juvenile polyposis and absence of BMPR2, BMPR1B, and ACVR1 mutations. J. Med. Genet. 41, 484–491 (2004).

    Article  CAS  Google Scholar 

  17. Arnold, R. Diagnosis and differential diagnosis of hypergastrinemia. Wien. Klin. Wochenschr. 119, 564–569 (2007).

    Article  CAS  Google Scholar 

  18. Grabowska, A. M. & Watson, S. A. Role of gastrin peptides in carcinogenesis. Cancer Lett. 257, 1–15 (2007).

    Article  CAS  Google Scholar 

  19. Rehfeld, J. F., Friis-Hansen, L., Goetze, J. P. & Hansen, T. V. The biology of cholecystokinin and gastrin peptides. Curr. Top. Med. Chem. 7, 1154–1165 (2007).

    Article  CAS  Google Scholar 

  20. Rehfeld, J. F. et al. Prohormone convertases 1/3 and 2 together orchestrate the site-specific cleavages of progastrin to release gastrin-34 and gastrin-17. Biochem. J. 415, 35–43 (2008).

    Article  CAS  Google Scholar 

  21. Rehfeld, J. F. The endoproteolytic maturation of progastrin and procholecystokinin. J. Mol. Med. 84, 544–550 (2006).

    Article  CAS  Google Scholar 

  22. Dockray, G. J., Varro, A., Dimaline, R. & Wang, T. The gastrins: their production and biological activities. Annu. Rev. Physiol. 63, 119–139 (2001).

    Article  CAS  Google Scholar 

  23. Hilsted, L. & Rehfeld, J. F. Measurement of precursors for alpha-amidated hormones by radioimmunoassay of glycine-extended peptides after trypsin-carboxypeptidase B cleavage. Anal. Biochem. 152, 119–126 (1986).

    Article  CAS  Google Scholar 

  24. Houli, N. et al. Mitogenic effects of both amidated and glycine-extended gastrin-releasing peptide in defunctioned and azoxymethane-treated rat colon in vivo. Regul. Pept. 134, 9–16 (2006).

    Article  CAS  Google Scholar 

  25. Patel, O. et al. Stimulation of proliferation and migration of a colorectal cancer cell line by amidated and glycine-extended gastrin-releasing peptide via the same receptor. Biochem. Pharmacol. 68, 2129–2142 (2004).

    Article  CAS  Google Scholar 

  26. Kopin, A. S. et al. Expression cloning and characterization of the canine parietal cell gastrin receptor. Proc. Natl Acad. Sci. USA 89, 3605–3609 (1992).

    Article  CAS  Google Scholar 

  27. Rehfeld, J. F. Accurate measurement of cholecystokinin in plasma. Clin. Chem. 44, 991–1001 (1998).

    CAS  PubMed  Google Scholar 

  28. Castell, D. O. Gastrin and lower esophageal sphincter tone. Arch. Intern. Med. 138, 196 (1978).

    Article  CAS  Google Scholar 

  29. Shemerovskii, K. A. & Ovsiannikov, V. I. Effect of pentagastrin on the myoelectric activity of the pyloric sphincter, body of the stomach and the duodenum. Fiziol. Zh. SSSR. Im. I. M. Sechenova. 72, 458–465 (1986).

    CAS  PubMed  Google Scholar 

  30. Ishiguchi, T. et al. Gastric distension-induced pyloric relaxation: central nervous system regulation and effects of acute hyperglycaemia in the rat. J. Physiol. 533, 801–813 (2001).

    Article  CAS  Google Scholar 

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Acknowledgements

Written consent for publication was obtained from the patient. The authors acknowledge the excellent support provided by Heidi Paulsen, Lise Strange and Grazyna Poulsen with regard to immunohistochemical stainings and artwork.

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Authors and Affiliations

  1. Department of Medicine, Division of Gastroenterology, University of Calgary, 3280 Hospital Drive NW, Calgary, T2N 4N1, Alberta, Canada

    Karen D. Papay, Remo Panaccione & Martin A. Storr

  2. Department of Pathology and Laboratory Medicine, University of Calgary, 3280 Hospital Drive NW, Calgary, T2N 4N1, Alberta, Canada

    Vincent G. Falck

  3. Department of Biomedical Sciences, The Panum Institute, University of Copenhagen, Ringshospitalet, DK-2010, Copenhagen, Denmark

    Steen S. Poulsen

  4. Department of Clinical Biochemistry, Faculty of Health Sciences, University of Copenhagen, Ringshospitalet, DK-2010, Copenhagen, Denmark

    Jens F. Rehfeld

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  1. Karen D. Papay
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Contributions

All authors researched data for the article and contributed equally to reviewing and/or editing of the manuscript before submission. K. D. Papay and M. A. Storr wrote the article. K. D. Papay, S. S. Poulsen, R. Panaccione, J. F. Rehfeld and M. A. Storr provided a substantial contribution to the discussion of content.

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Correspondence to Martin A. Storr.

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The authors declare no competing financial interests.

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Papay, K., Falck, V., Poulsen, S. et al. Juvenile polyposis of the stomach—a novel cause of hypergastrinemia. Nat Rev Gastroenterol Hepatol 7, 583–588 (2010). https://doi.org/10.1038/nrgastro.2010.138

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