Clinical classification of symptomatic heterotopic pancreas of the stomach and duodenum: A case series and systematic literature review

Abstract

BACKGROUND Heterotopic pancreas (HP) is an aberrant anatomic malformation that occurs most commonly in the upper gastrointestinal tract. While the majority of heterotopic pancreatic lesions are asymptomatic, many manifest severe clinical symptoms which require surgical or endoscopic intervention. Understanding of the clinical manifestations and symptoms of HP is limited due to the lack of large volume studies in the literature. The purpose of this study is to review symptomatic cases at a single center and compare these to a systematic review of the literature in order to characterize common clinical manifestations and treatment of this disease. AIM To classify the common clinical manifestations of heterotopic pancreas. METHODS A retrospective review was conducted of pathologic samples containing heterotopic pancreas from 2000-2018. Review was limited to HP of the upper gastrointestinal tract due to the frequency of presentation in this location. Symptomatic patients were identified from review of the medical records and clinical symptoms were tabulated. These were compared to a systematic review of the literature utilizing PubMed and Embase searches for papers pertaining to heterotopic pancreas. Publications describing symptomatic presentation of HP were selected for review. Information including demographics, symptoms, presentation and treatment were compiled and analyzed. RESULTS Twenty-nine patient were identified with HP at a single center, with six of these identified has having clinical symptoms. Clinical manifestations included, gastrointestinal bleeding, gastric ulceration with/without perforation, pancreatitis, and gastric outlet obstruction. Systemic review of the literature yielded 232 publications detailing symptomatic cases with only 20 studies describing ten or more patients. Single and multi-patient studies were combined to form a cohort of 934 symptomatic patients. The majority of patients presented with abdominal pain (67%) combined with one of the following clinical categories: (1) Dyspepsia, ( n = 445, 48%); (2) Pancreatitis ( n = 260, 28%); (3) Gastrointestinal bleeding ( n = 80, 9%); and (4) Gastric outlet obstruction ( n = 80, 9%). The majority of cases ( n = 832, 90%) underwent surgical or endoscopic resection with 85% reporting resolution or improvement in their symptoms. CONCLUSION Heterotopic pancreas can cause significant clinical symptoms in the upper gastrointestinal tract. Better understanding and classification of this disease may result in more accurate identification and treatment of this malformation.

Gastric Hematoma Secondary to Heterotopic Pancreas of the Stomach: Unexpected Cause of Upper Gastrointestinal Bleeding

“…HP is an uncommon embryologic abnormality and is defined as aberrant pancreatic tissue separated anatomically from the main body and blood supply of the pancreas [ 4 ]. HP can have either exocrine or endocrine tissue, or both.…”

“…HP is considered uncommon with reported rates ranging from as low as 0.6% in endoscopic studies to as high as 16% in autopsy studies [ 4 , 5 ]. There are slightly more men affected (61%) and the mean age of symptoms presentation was reported to be 43 years [ 4 ]. HP can be found in any part of the gastrointestinal tract.…”

“…Up to 75% are found in the stomach, duodenum, and ileum, with stomach accounting for between 25% and 37% [ 5 ]. In a large series of 934 symptomatic patients, 59% of lesions were located in the stomach and 41% in the duodenum [ 4 ]. Generally, for stomach HP, 90% are located on the greater curvature of the antrum within 6 cm from the pylorus, appearing as a mucosal bulge with a central umbilication or punctum [ 6 ].…”

“…Most HP, regardless of the location are asymptomatic and are diagnosed incidentally. However, some can manifest with clinical symptoms and complications such as pancreatitis, gastric outlet obstruction, and even malignant transformation [ 4 , 5 , 7 , 8 ]. In a large single-center series that included 29 patients with upper gastrointestinal HP, only six (20.9%) had clinical symptoms [ 4 ].…”

“…However, some can manifest with clinical symptoms and complications such as pancreatitis, gastric outlet obstruction, and even malignant transformation [ 4 , 5 , 7 , 8 ]. In a large single-center series that included 29 patients with upper gastrointestinal HP, only six (20.9%) had clinical symptoms [ 4 ]. However, a literature review of 232 publications (N=1762 patients) identified 934 (53%) patients with gastric or duodenal HP who had reported symptoms [ 4 ].…”