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Heterotopic Pancreas in the Esophagus: What Do We Know?—A Review of the Literature

Open AccessPublished:July 18, 2022DOI:https://doi.org/10.1016/j.gastha.2022.06.010
      Heterotopic pancreatic tissue is a rare phenomenon, which may occur in locations outside of the pancreas within the gastrointestinal tract. Of these locations, involvement in the esophagus is quite rare. It is paramount to improve our understanding regarding heterotopic pancreas, as some lesions may carry malignant potential. With this article, we present a case of heterotopic pancreas in the distal esophagus and review the current literature.

      Keywords

      Introduction

      Heterotopic pancreas (HP) is a rare congenital malformation, which is usually discovered incidentally. It has been previously noted in various parts of the gastrointestinal tract; however, involvement in the esophagus is rare.

      Case Report

      Patient is a 59-year-old female with a past medical history of asthma, narcolepsy, prediabetes, and pancreatitis who presented with right upper quadrant pain radiating to the shoulder and nausea. Patient denied any hematemesis, melena, dysphagia or odynophagia, change in bowel movements, or weight loss. She denied any non-steroidal anti-inflammatory drugs or blood thinner use. Routine blood work was unremarkable. Esophagogastroduodenoscopy was performed, which revealed grade A esophagitis (Figure 1).
      Figure thumbnail gr1
      Figure 1(A, B) EGD showing area of distal esophagus with visible evidence of inflammation and grade A esophagitis.
      Subsequent tissue biopsy revealed evidence of active reflux esophagitis and granulation tissue consistent with focal pancreatic heterotopia (Figure 2).
      Figure thumbnail gr2
      Figure 2(A, B) Histopathology demonstrating foci of acinar pancreatic tissue with surrounding inflammatory cells in the background of distal esophageal squamocolumnar epithelium.
      Given the patient’s active inflammation, she was treated with proton pump inhibitor with improvement in symptoms. Patient was told to continue routine surveillance of her esophagitis.

      Discussion

      HP is a congenital malformation defined as pancreatic tissue that is not anatomically or vascularly connected to the normal pancreatic body.
      • Eisenberger C.F.
      • et al.
      HP can occur in any part of the digestive tract, but it is most commonly found in the stomach (30%), the duodenum (17%–36%), and the jejunum (15%–21%).
      • Eisenberger C.F.
      • et al.
      It is most often diagnosed incidentally on autopsies with a prevalence between 0.5% and 13% and is more commonly found in men.
      • Shalaby M.
      • et al.
      There are several theories proposed regarding the pathogenesis of HP, including the totipotent cell theory, the pancreatic metaplasia theory, and misplacement theory. The totipotent cell theory assumes that the endodermal cells of the bowel wall have the potential to differentiate into pancreatic tissue. The pancreatic metaplasia theory stipulates that during embryogenesis, pancreatic tissue migrates to intestinal submucosa. The misplacement theory assumes that pancreatic tissue is randomly lost throughout the developing primitive foregut.
      • Shalaby M.
      • et al.
      Although heterotopic pancreatic tissue may not be in close proximity to the pancreas, it is susceptible to similar pathology, such as acute or chronic inflammation.
      • Rezvani M.
      • et al.
      Several reports have demonstrated pseudocyst and fibrotic features of heterotopic pancreatic tissue. Furthermore, evidence of premalignant lesions and adenocarcinoma has also been identified, although most cases were from stomach and duodenum.
      • Rezvani M.
      • et al.
      HP in the esophagus is a rare entity. It has previously been reported only 17 times in the medical literature
      • Temes R.T.
      • et al.
      • Lowry D.M.
      • et al.
      • Crighton E.
      • et al.
      • Goto J.
      • et al.
      • Ulrych J.
      • et al.
      • Gananadha S.
      • et al.
      • Salo J.A.
      • et al.
      • Roshe J.
      • et al.
      • Razi M.D.
      • et al.
      • Shalaby M.
      • et al.
      • Guillou L.
      • et al.
      • Garn T.
      • et al.
      • Salim S.T.
      • et al.
      • Rodriguez F.J.
      • et al.
      • Shamoon A.
      • et al.
      • Filip R.
      • et al.
      (Table).
      TableReview of Cases of Heterotopic Pancreas in the Esophagus Including Patient Symptomatology, Location of Lesion, Management of Lesion (if Provided) and Malignant Potential
      CaseAgeSexSymptomsLocationManagementMalignancyReference
      124FChest pain, vomiting, shortness of breathDistal esophagusEsophageal enucleation procedureNoneTemes et al
      • Temes R.T.
      • et al.
      225MRUQ abdominal painDistal esophagusVATSNoneLowry et al
      • Lowry D.M.
      • et al.
      358FWorsening dysphagiaDistal esophagusThorascopic ILEIPMNCrighton et al
      • Crighton E.
      • et al.
      447FVomiting, epigastric painDistal esophagusILE, Witzel jejunostomy with pyroloplastyNoneNoffsinger et al
      563MAsymptomaticMiddle esophagusNo surgical intervention, conservativeNoneGoto et al
      • Goto J.
      • et al.
      634MWorsening odynophagia and dysphagiaDistal esophagusPartial fundoplicationNoneUlrych et al
      • Ulrych J.
      • et al.
      726FNausea and epigastric painDistal esophagusLaporascopic excision followed by Partial Dor fundoplicationNoneGananadha et al
      • Gananadha S.
      • et al.
      825MVomiting and abdominal painDistal esophagusEnucleation via right thoractomyNoneSalo et al
      • Salo J.A.
      • et al.
      945MProgressive dysphagiaDistal esophagusThoracoabdominal esophagogastrectomyAnaplastic cancerRoshe et al
      • Roshe J.
      • et al.
      1043MUpper GI bleedingDistal esophagus/lesser curvature of stomachEnucleation of tumor from esophagusNoneRazi et al
      • Razi M.D.
      • et al.
      1152MIntermittent dysphagiaDistal esophagusNo surgical intervention, conservativeNoneShalaby et al
      • Shalaby M.
      • et al.
      1260MDysphagia and epigastric painDistal esophagusTumor resection with proximal stomach resectionAdenocarcinomaGuillou et al
      • Guillou L.
      • et al.
      1338FHeartburn and nauseaDistal esophagusEndoscopically assisted laparoscopic resection of tumorNoneGarn et al
      • Garn T.
      • et al.
      1429MDysphagia and epigastric painDistal esophagusNo treatment mentionedNoneSalim et al
      • Salim S.T.
      • et al.
      1541FDysphagia and epigastric painDistal esophagus/lesser curvature of stomachNo treatment mentionedNoneRodriguez et al
      • Rodriguez F.J.
      • et al.
      1670MHeartburn, nausea and abdominal bloatingDistal esophagusEndoscopic resectionNoneShamoon et al
      • Shamoon A.
      • et al.
      1773FEpigastric pain, vomitingDistal esophagusEndoscopic resectionNoneFilip et al
      • Filip R.
      • et al.
      GI, gastrointestinal; ILE, ileoscopy; RUQ, right upper quadrant; VATS, video assisted thorascopic surgery.
      Of the 17 cases reported, 16 of them were found in the distal esophagus/gastroesophageal junction. Although the condition is typically asymptomatic, it can cause epigastric pain, dysphagia, and upper gastrointestinal bleeding. Based on previous reports that have suggested the presence of malignancy in some of these lesions, adequate stratification of patients into those who are surgical candidates vs those who could have active surveillance is necessary. Furthermore, given that most of the previous cases underwent surgical or endoscopic intervention, there are no clear guidelines on the management of patients with asymptomatic and benign cases such as our case. Should these patients receive antireflux therapy? Should they undergo surveillance endoscopy every 1–3 years? Should the lesion be pre-emptively excised given potential for pre-malignancy? These questions will need to be answered eventually to better serve patients with pancreatic heterotypia.

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