Display options
Share it on

Cancers (Basel). 2012 Feb 14;4(1):130-40. doi: 10.3390/cancers4010130.

Primary hepatic gastrinoma causing zollinger-ellison syndrome: a rare and challenging diagnosis.

Cancers

Adrian Harvey, Janice L Pasieka, Hassan Al-Bisher, Elijah Dixon

Affiliations

  1. Division of General Surgery and Surgical Oncology, Department of Surgery and Oncology, University of Calgary, Calgary, Alberta T2N 2T9, Canada. [email protected].

PMID: 24213231 PMCID: PMC3722648 DOI: 10.3390/cancers4010130

Abstract

The majority of gastrinomas causing Zollinger-Ellison syndrome (ZES) are located in the duodenum or the pancreas. Primary hepatic gastrinomas (PHG) are extremely rare and difficult to diagnose because the liver is the commonest site of metastatic disease and gastrinomas can be very small. Furthermore, gastrinomas are typically slow-growing thus a missed, occult primary tumour may not become evident for many years. The diagnosis of PHG is therefore dependent on a careful search for a primary and long-term biochemical follow-up following curative hepatic resection. We report a case of a 7 cm PHG in a 48 year old man with ZES. Preoperatively, both a basal and stimulated gastrin levels were elevated. Surgical exploration including intraoperative ultrasound and duodenotomy, failed to reveal a primary. Patient underwent a right hepatectomy. Yearly, gastrin and secretin stimulation tests remain normal 6 years following surgery. He remains symptom free off all medication. An additional 26 cases of PHG were found. Including this case, 21 had at least 1 year follow-up, however only eight had greater than 5 years (median 24 months). Post-op gastrin levels were reported in 25, however provocative testing was done in only 10. Persistence and recurrence occurred in one and four, respectively. PHG causing ZES is extremely rare. Although the current literature claims to include 26 additional cases of PHG, without a thorough search for the primary and long-term follow-up data including provocative testing, this diagnosis remains a challenge.

References

  1. Br J Radiol. 1999 Feb;72(854):207-9 - PubMed
  2. Ultrastruct Pathol. 1986;10(4):331-6 - PubMed
  3. Dig Dis Sci. 2003 Aug;48(8):1665-7 - PubMed
  4. Cancer. 1990 Mar 1;65(5):1211-8 - PubMed
  5. Ann Surg. 2006 Sep;244(3):410-9 - PubMed
  6. Surgery. 1998 Dec;124(6):1145-52 - PubMed
  7. Ann Intern Med. 1993 Aug 1;119(3):199-206 - PubMed
  8. Ann Oncol. 1999;10 Suppl 4:182-4 - PubMed
  9. Ultrastruct Pathol. 1992 Nov-Dec;16(6):667-72 - PubMed
  10. Dig Dis Sci. 2001 Feb;46(2):370-5 - PubMed
  11. Cancer. 1993 Sep 1;72(5):1547-50 - PubMed
  12. Hepatogastroenterology. 2008 Nov-Dec;55(88):2224-7 - PubMed
  13. Br J Surg. 2007 Nov;94(11):1331-41 - PubMed
  14. Dig Dis Sci. 2006 Jun;51(6):1122-5 - PubMed
  15. Am J Gastroenterol. 1999 Nov;94(11):3380-2 - PubMed
  16. Indian Pediatr. 1994 Sep;31(9):1137-40 - PubMed
  17. World J Surg. 1998 Jul;22(7):643-9; discussion 649-50 - PubMed
  18. Am J Obstet Gynecol. 2004 Nov;191(5):1716-8 - PubMed
  19. J Clin Gastroenterol. 1997 Apr;24(3):188-91 - PubMed
  20. J Korean Med Sci. 2010 Jun;25(6):953-6 - PubMed
  21. N Engl J Med. 1982 Jun 24;306(25):1533-6 - PubMed
  22. Gastroenterol Hepatol (N Y). 2010 Jan;6(1):53-6 - PubMed
  23. J Surg Oncol. 1996 Jul;62(3):218-21 - PubMed
  24. Surgery. 1985 Dec;98(6):1113-20 - PubMed
  25. Ultrastruct Pathol. 1983 Jul;5(1):55-72 - PubMed
  26. Ann Surg. 1955 Oct;142(4):709-23; discussion, 724-8 - PubMed
  27. J Pediatr Gastroenterol Nutr. 1984 Nov;3(5):801-4 - PubMed
  28. Eur J Surg. 1992 Jan;158(1):55-7 - PubMed
  29. Ann Surg. 2003 May;237(5):650-7; discussion 657-9 - PubMed
  30. World J Gastrointest Pathophysiol. 2011 Apr 15;2(2):26-30 - PubMed
  31. Medicine (Baltimore). 2006 Nov;85(6):331-364 - PubMed
  32. Cases J. 2009 Aug 07;2:6346 - PubMed
  33. BMJ Case Rep. 2009;2009: - PubMed

Publication Types