An Unusual Location for Pancreatic Heterotopia: The Gastroesophageal Junction

Abstract

: Heterotopic pancreas (HP) is defined as pancreatic tissue found outside the pancreas. When symptoms appear, they are often due to inflammation, obstruction, and malignant transformation. The most common location is the stomach, especially the antrum. HP in the esophagus is uncommon, with only a handful of cases reported in pediatric patients. It rarely occurs in the adult population.We present an atypical case of HP at the gastroesophageal junction (GE) in a middleaged female. Case: A 59 year old African American female was referred for iron deficiency anemia. Patient had recurrent cough. She denied symptoms of dyspepsia. Physical exam was unremarkable. The white blood cell was 9.7 (3.8-10.6), blood Hg 11.9 g/dL, HCT 34.4% with an MCV of 77.8 L, and platelet count was 246. Stool occult testing negative. An esophagogastroduodenoscopy revealed a small, broad-based polyp about 3-4 mm in diameter without umbilication, or evidence of bleeding (Figure 1). It was glimpsed at the GE junction on introduction of the endoscope, and confirmed on a retroflexed view (Figure 2). A biopsy of the polyp was obtained with cold forceps, and sent for histopathologic examination which revealed focal pancreatic acinar cell metaplasia/heterotopia completely surrounded by gastric cardia-type mucosa with chronic active gastritis, acute cryptitis (Figure 3). There was no evidence of intestinal metaplasia or dysplasia, or esophageal squamous mucosa. Because the polyp was located at the GE junction, the origin could have been esophageal or gastric. However, because the surrounding tissue histologically was entirely gastric, a diagnosis of HP of the stomach at the GE junction was assigned. The patient had been asymptomatic and did not present with complications, a follow up plan for endoscopic surveillance was proposed.Figure 1Figure 2Figure 3Discussion: The incidence of HP cases has been reported to be 2 to 15%, with the majority found incidentally during surgery or post-mortem autopsies. Endoscopically, HPs grossly appear as firm white to yellow lobulated nodules and range in size almost 0.2 to 4 cm. Our case was unusual in that it did not have the characteristic umbilication, nor discoloration, but rather a nodular polypoid appearance, and was located not in the atrum or body, but at the GE junction. In a symptomatic patient, resection is performed after other causes are ruled out. In an asymptomatic patient, with benign gross and histological features, lesions are managed conservatively.