This patient with multiple, severe ulcers from his stomach to his small bowel most likely had Zollinger-Ellison syndrome (ZES), which is caused by a gastrin-secreting tumor.
ZES typically presents with chronic diarrhea, nausea, abdominal pain, and weight loss. It is caused by a gastrin-secreting tumor of the pancreas or duodenum that results in hyper-secretion of gastric acid. Patients with ZES often have multiple or refractory ulcers, as well as ulcers in unusual locations (e.g. distal duodenum or jejunum). Approximately 25% of patients with ZES have multiple endocrine neoplasia syndrome 1 (MEN1), a genetic syndrome causing pituitary, parathyroid, and pancreatic tumors. This patient, who had a history of recurrent nephrolithiasis, may have had an undiagnosed parathyroid adenoma causing hypercalcemia.
Ramakrishnan and Salinas review the presentation, diagnosis, and management of peptic ulcer disease. In particular, they note that ZES is one of the very few causes of peptic ulcers in the esophagus, distal duodenum, or jejunum (the others being hiatal hernias for esophageal or ectopic gastric mucosa for small bowel). Esophagogastroduodenoscopy (EGD) is indicated for any patient suspected of having peptic ulcers that is >55yo, has evidence of bleeding, weight loss, chronicity, persistent nausea, or is refractory to treatment.
Norton et al. performed a prospective case control study looking at whether patients with sporadic ZES should undergo surgery if they have negative imaging studies such as somatostatin receptor scintigraphy. They found that experienced surgeons have rates of finding the primary tumor comparable to those in patients with positive imaging results, and that the risk of metastasis is high enough to justify early surgical intervention.
Illustration A shows the feedback loop that controls acid secretion in the stomach.
Illustration B is from an EGD and shows the distal duodenum of a patient with ZES filled with multiple small ulcers.
Answer 2: VIP-secreting tumors may occur in MEN1, and often present with nausea, diarrhea, and abdominal pain. However, VIP suppresses gastric acid production, and peptic ulcers are not typically present.
Answer 3: Cytomegalovirus is associated with peptic ulcer formation, but does not typically cause multiple or jejunal ulcers.
Answer 4: H. pylori is one of the leading causes of peptic ulcers, but does not typically cause ulcers reaching into the jejunum.
Answer 5: Chronic NSAID use typically causes gastritis. Though it can cause peptic ulcer disease, it would not explain the severity of this patient's disease.
Ramakrishnan K, Salinas RC. Peptic ulcer disease. Am Fam Physician. 2007 Oct 1;76(7):1005-12. Review.
PMID:17956071 (Link to Abstract)
Norton JA, Fraker DL, Alexander HR, Jensen RT. Value of surgery in patients with negative imaging and sporadic Zollinger-Ellison syndrome. Ann Surg. 2012 Sep;256(3):509-17.
PMID:22868363 (Link to Abstract)
USMLE World Step 1 QBank Question #403. Copyright © USMLEWorld, LLC 2012