A 60-year-old woman presented to our hospital with severe nausea, vomiting, watery diarrhea, and burning epigastric pain for a duration of one week. Her epigastric pain was associated with severe acid reflux, which had been intermittently present for a duration of two years and was resistant to over-the-counter low-dose proton pump inhibitor (PPI) therapy. Her past medical history was negative for any evidence of gastrointestinal (GI) bleed. Interestingly, the patient had a daughter who had been diagnosed with multiple endocrine neoplasia (MEN) type 1 a year prior to presentation. On physical exam, she was afebrile with stable hemodynamics. Abdominal palpation revealed mild epigastric tenderness without any guarding or rigidity. Cardiopulmonary exam was within normal limits.
Significant laboratory findings included WBC count of 15,000/microL, potassium of 3 mmol/L, magnesium of 0.7 mg/dL, and calcium of 11.8 mg/dL. Lipase level was within normal limits. Other pertinent laboratory values included fasting serum gastrin level of 1603 pg/mL (0-180 pg/mL), chromogranin A level of 14600 ng/mL (0-100 ng/mL), prolactin hormone level of 21 ng/mL (2-29 ng/mL), and parathyroid hormone (PTH) level of 473 pg/mL (10-65 pg/mL). She did not have any history of prior gastric surgeries, gastroparesis, or renal disease, to possibly explain her elevated gastrin level. An infectious workup for her diarrhea, including Clostridium difficile toxin and a stool PCR panel for common enteric pathogens, was negative. Subsequently, an extensive workup for evaluation of MEN was done, which revealed a unilateral parathyroid adenoma on neck imaging and diffuse stomach wall thickening along with pancreatic cystic lesions in body (1.2 cm) and tail (0.7 cm) on abdominal MRI (Figure 1). Testing for pituitary disease was negative.
An esophagogastroduodenoscopy (EGD) was performed for further evaluation of her symptoms, which revealed severe reflux esophagitis, diffusely hypertrophic gastric rugae and multiple postbulbar ulcers in the duodenum (Figures 2(a), 2(b), and 2(c)). Endoscopic ultrasound (EUS) subsequently revealed diffuse thickening of the gastric rugae, predominantly of echo-layers I-III (Figure 3(a)). In addition, the patient was found to have a cystic lesion in the pancreatic neck with thick hypoechoic walls (Figure 3(b)). Random biopsies of the gastric antrum and body revealed patchy chronic gastritis with intestinal metaplasia (Figure 4(a)) while FNA from pancreatic cyst revealed well differentiated NET (Figure 4(b)).
The patient eventually underwent a distal pancreatectomy and parathyroidectomy with clinical improvement. The remainder of her hospital course was uncomplicated and she was discharged home on high-dose PPI and octreotide.