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Clinical Features: Pancreatic heterotopia is a congenital lesion; however, most symptomatic cases are detected in adulthood (average age 45 years). About 50-75% of patients have no symptoms.

Patients can develop abdominal discomfort or pain, nausea, vomiting, and gastrointestinal bleeding. Large lesions in prepyloric region can cause gastric outlet obstruction. Intussusception has resulted in some cases. The heterotopic tissue can also develop lesions typical of pancreas, including pancreatitis, cysts, neuroendocrine tumors, or ductal adenocarcinoma.

Endoscopic & Gross Appearance: Pancreatic heterotopia usually presents as solitary mass ranging in size from 0.5 to 5 cm. Gastric lesions are located in the antrum or prepyloric region. The duodenal cases involve the second portion several centimeters proximal to the ampulla of Vater.

At endoscopy, it appears as a dome-shaped, intramural nodule with smooth overlying mucosa. A central umbilication or dimple marks the site of draining duct. On sectioning, it is a well-circumscribed, yellowish, lobulated nodule resembling normal pancreas, located in the submucosa or muscularis propria.

Image courtesy of Dr. Jean-Christophe Fournet, Paris, France; humpath.com; Used with permission

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