Published online Apr 14, 2022. doi: 10.3748/wjg.v28.i14.1455
Peer-review started: June 8, 2021
First decision: June 27, 2021
Revised: July 11, 2021
Accepted: March 4, 2022
Article in press: March 4, 2022
Published online: April 14, 2022
Processing time: 302 Days and 6.4 Hours
Heterotopic pancreas (HP) is a rare embryologic anomaly that occurs when aberrant pancreatic tissue develops independently from the main body of the main body of the pancreas. This can occur throughout the body and develop in various tissues however, this most commonly occurs in the stomach and duodenum. While the majority of these lesions are asymptomatic, many have the potential to cause harmful clinical manifestations.
Symptomatic heterotopic pancreas is difficult to identify and often mimics common upper gastrointestinal pathology making diagnosis and treatment challenging. Symptomatic cases of HP often require surgical or endoscopic management however, the optimal treatment strategy is poorly understood at this time and patients often undergo radical resection.
The purpose of this study was to review the cases of symptomatic HP at a single, high volume academic institution and compare these with a cohort of patients derived from a systematic review of symptomatic cases in the English literature. This combined cohort was used to classify the common presentation and clinical symptoms for HP and identify the various treatment options.
A retrospective review was conducted of pathologic specimens containing heterotopic pancreas at a single, high volume, academic medical center. Symptomatic cases were identified and analyzed for details related to presentation, symptoms and treatment. A systematic review was then conducted utilizing Embase and PubMed databases for publications in English which pertained to heterotopic pancreas. Publications which included symptomatic cases of HP were included for final literature analysis and results were tabulated into a final cohort for review.
Institutional review: Twenty-nine cases of HP were identified at a single institution, of which, six presented with symptoms (20%). Two patients (7%) presented with elevated lipase and evidence of pancreatitis within the heterotopic lesion, two patients presented with gastrointestinal bleeding or anemia, one patient (3.4%) presented with perforated gastric ulceration related to a heterotopic lesion, and one patient presented with gastric outlet obstruction from a HP lesion located at the pylorus.
Systematic review: Literature review resulted in identification of 1220 unique publications related to HP of which 232 publications met inclusion criteria and were reviewed. The final cohort of patients was 934 patients. Analysis of symptomatic cases revealed that the majority of cases could be classified into the following: (1) Dyspepsia, which included symptoms of epigastric pain, eructation, nausea or bloating with meals (n = 445, 48%); (2) Pancreatitis within the ectopic lesion (n = 260, 28%); (3) Gastrointestinal bleeding (n = 80, 9%); and (4) Gastric outlet obstruction (n = 80, 9%). 37 patients presented with jaundice and biliary obstruction due to periampullary lesions (4%), and 3 patients presented with perforated ulcers. The majority of patients underwent surgical or endoscopic resection (n = 832, 90%) and 85% of reported cases indicated symptom resolution. However, most patients underwent some form of formal anatomic resection (n = 490, 61%) most commonly related to improper diagnosis preoperatively and few benefited from local surgical or endoscopic resection.
Heterotopic pancreas is a rare condition but has the ability to manifest severe clinical symptoms. These most commonly arise in the stomach and duodenum and can be classified within for main categories. Improved recognition and diagnosis of this disease process may help guide treatment recommendations and improve outcomes for patients.
Prospective evaluation of medical and interventional therapies is needed to determine the most appropriate treatment for the different manifestations for heterotopic pancreas.