A 46-year-old woman comes to the office with persistent diarrhea, weight loss, and abdominal pain. Her diarrhea started several months ago. She has no fever, melena, or hematochezia. The patient has not traveled outside of the country and has not drunk from any untreated freshwater sources. She has had no recent hospitalizations or antibiotic use. Her sister has a history of celiac disease. Upper endoscopy reveals postbulbar duodenal and jejunal ulcers. This patient most likely has a tumor secreting which of the following molecules?

Question

A 46-year-old woman comes to the office with persistent diarrhea, weight loss, and abdominal pain.  Her diarrhea started several months ago.  She has no fever, melena, or hematochezia.  The patient has not traveled outside of the country and has not drunk from any untreated freshwater sources.  She has had no recent hospitalizations or antibiotic use.  Her sister has a history of celiac disease.  Upper endoscopy reveals postbulbar duodenal and jejunal ulcers.  This patient most likely has a tumor secreting which of the following molecules?

Quizplus · Accepted Answer

11ec160b_3967_fbc9_9249_2faba0bb10e4_MD0001_00 This patient's clinical presentation is highly suggestive of Zollinger-Ellison syndrome (ZES). ZES is caused by gastrin-secreting neuroendocrine tumors (gastrinomas) that are most commonly located in the small intestine or pancreas. The gastrin produced by these tumors stimulates gastric acid production, often resulting in multiple peptic ulcers that can be located beyond the duodenal bulb and are often refractory to therapy. Patients typically experience abdominal pain and acid reflux. They may also have diarrhea as gastric acid damages intestinal epithelial cells and inactivates pancreatic enzymes, preventing proper nutrient absorption. The diagnosis of gastrinoma is made by measuring basal and stimulated gastrin levels (secretin stimulation test). Patients with ZES should undergo testing (eg, serum calcium, prolactin level) to exclude multiple endocrine neoplasia type 1 due to the strong association between these conditions. (Choice B) Glucagonomas are rare pancreatic alpha-cell tumors that present with diabetes mellitus and a raised erythematous rash that typically affects the groin (necrolytic migratory erythema). (Choice C) Insulinomas are pancreatic beta-cell tumors that secrete insulin autonomously, regardless of blood glucose levels. As a result, patients usually have high circulating insulin/C-peptide levels and symptoms of hypoglycemia (eg, tremor, confusion). (Choice D) Carcinoid syndrome is most frequently caused by neuroendocrine tumors originating in the small intestine that have metastasized to the liver. These tumors secrete products such as serotonin and bradykinin, resulting in diarrhea, episodic flushing, right-sided valvular heart disease, and wheezing. (Choice E) Somatostatinomas are rare tumors of the pancreatic D cells that present with diabetes mellitus (diminished insulin secretion), cholelithiasis (inhibition of cholecystokinin release), and diarrhea/steatorrhea (inhibition of pancreatic enzyme/bicarbonate secretion). (Choice F) Vasoactive intestinal polypeptide is secreted by VIPomas, rare neuroendocrine tumors of the pancreas. Patients typically have diarrhea that persists with fasting, achlorhydria, and hypokalemia. Educational objective: Zollinger-Ellison syndrome is caused by gastrin-secreting tumors (gastrinomas) involving the small intestine or pancreas. Patients typically have peptic ulcers (often beyond the duodenal bulb), abdominal pain/acid reflux, and diarrhea. The condition is frequently associated with multiple endocrine neoplasia type 1. __________ References: Gastrinoma Zollinger-Ellison-syndrome. (http://www.ncbi.nlm.nih.gov/pubmed/25905301) Hypergastrinemia. (http://www.ncbi.nlm.nih.gov/pubmed/25698559)

A 46-Year-Old Woman Comes to the Office with Persistent Diarrhea