• Heterotopic pancreas
• benign stricture
• congenital malformation
• dysphagia
• esophagus

Heterotopic pancreas is a rare congenital abnormality that occurs during the growth and development process. It can be found in any part of the digestive tract, but the most common sites are the stomach, duodenum, and jejunum. Malignant transformation especially in the esophagus is rare. Here, we aim to report an unusual case of mid‐esophageal adenocarcinoma that originated from a heterotopic pancreas.

Heterotopic pancreas, commonly referred to as pancreatic rest or ectopic pancreas, is a congenital anomaly in which pancreatic tissue is anatomically separate from the main pancreatic gland without continuity of a duct system or vascularity. It is commonly found in the upper gastrointestinal tract, specifically in the stomach and small intestine. To date, only about 18 adult cases of heterotopic pancreas in the esophagus have been reported in the English medical literature; seven of which were in women, and five of which originated at the gastroesophageal junction (GEJ). Of these five cases, only two occurred in a hiatal hernia. We report the third case of the heterotopic pancreas at the GEJ in a hiatal hernia discovered in a 62-year-old Caucasian female who presented to the outpatient clinic complaining of worsening reflux characterized as burning retrosternal chest pain. The patient failed maximum medical therapy and was referred to general surgery for laparoscopic hiatal hernia repair with Toupet fundoplication to prevent further reflux and damage to the esophagus. The reflux symptoms persisted after the procedure. Follow-up esophagogastroduodenoscopy with biopsy of the GEJ revealed a small focus of heterotopic pancreas tissue, confirmed by histopathology. The management of heterotopic pancreas differs throughout the literature depending on the size, symptomatology, and potential for malignancy. Management in cases of pancreatic rest, specifically at the GEJ, ranges from observation with conservative medical therapy, resection, or esophagectomy. With this case, we aim to contribute to the literature with the third case of pancreatic rest in the GEJ of a hiatal hernia.

We report a case of intraductal papillary mucinous neoplasm (IPMN) originating from the ileal heterotopic pancreas in a patient with hereditary non-polyposis colorectal cancer (HNPCC). A 49-year-old woman had a past history of total colectomy and total hysterectomy with bilateral salpingo-oophorectomy due to colonic adenocarcinoma and endometrial adenocarcinoma 11 years ago. Her parents died from colonic adenocarcinoma and her sister died from colonic adenocarcinoma and endometrial adenocarcinoma. The clinician found an ileal mass with necrotic change and the mass increased in size from 1.7 cm to 2.2 cm during the past 2 years on computed tomography. It was surgically resected. Microscopically, the ileal mass showed heterotopic pancreas with IPMN high grade dysplasia. Immunohistochemical staining revealed positive reactivity for MLH1/PMS2 and negative reactivity for MSH2/MSH6. This is the first report of IPMN originating from the ileal heterotopic pancreas in a patient with HNPCC in the English literature.

• Colorectal neoplasms
• Hereditary nonpolyposis
• Pancreatic neoplasms
• Choristoma

• Adenocarcinoma/genetics
• Adenocarcinoma/pathology
• Adenocarcinoma/surgery
• Biomarkers, Tumor/analysis
• Biopsy
• Choristoma
• Colectomy
• Colorectal Neoplasms, Hereditary Nonpolyposis/genetics
• Colorectal Neoplasms, Hereditary Nonpolyposis/pathology
• Colorectal Neoplasms, Hereditary Nonpolyposis/surgery
• Female
• Genetic Predisposition to Disease
• Heredity
• Humans
• Ileal Neoplasms/chemistry
• Ileal Neoplasms/pathology
• Ileal Neoplasms/surgery
• Immunohistochemistry
• Middle Aged
• Neoplasms, Cystic, Mucinous, and Serous/chemistry
• Neoplasms, Cystic, Mucinous, and Serous/pathology
• Neoplasms, Cystic, Mucinous, and Serous/surgery
• Pancreas
• Pancreatic Neoplasms
• Phenotype
• Tomography, X-Ray Computed
• Tumor Burden