Intraductal papillary mucinous neoplasm originating from a jejunal heterotopic pancreas:A case report

BACKGROUND Intraductal papillary mucinous neoplasm(IPMN)is a rare pancreatic tumor and has the potential to become malignant.Surgery is the most effective treatment at present,but there is no consensus on the site of resection.Heterotopic pancreas occurs in the gastrointestinal tract,especially the stomach and duodenum but is asymptomatic and rare.We report a case of ectopic pancreas with IPMN located in the jejunum.CASE SUMMARY A 56-year-old male patient suffered from severe pain,nausea and vomiting due to a traffic accident and sought emergency treatment at our hospital.Contrast-enhanced computed tomography of the whole abdomen suggested splenic congestion,which was considered to be splenic rupture.Emergency laparotomy was performed,and the ruptured spleen was removed during the operation.Unexpectedly,a cauliflower-like mass of about 2.5 cm×2.5 cm in size was incidentally found about 80 cm from the ligament of Treitz during the operation.A partial small bowel resection was performed,and postoperative pathology confirmed the small bowel mass as heterotopic pancreas with low-grade IPMN.CONCLUSION Ectopic pancreas occurs in the jejunum and is pathologically confirmed as IPMN after surgical resection.

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

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 Pub Med 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.

Heterotopic pancreas adenocarcinoma in the stomach: A case report and literature review

BACKGROUND Heterotopic pancreas is a common lesion found in the gastrointestinal tract and is usually considered a benign disease.Reports of malignant change of heterotopic pancreas are scarce.CASE SUMMARY A 44-year-old Chinese female underwent a gastroscopy to assess abdominal distension that had persisted for 2 months.A protruding lesion in the gastric antrum was revealed but no malignant tissue was found in the biopsy specimen.The patient's symptom persisted and progressed to repeated vomiting.Endoscopy after 4 months revealed obstruction of the gastric outlet caused by the protruding lesion.A distal gastrectomy was performed.Histopathological examination of the surgical specimen showed the malignant transformation of aberrant pancreas in the stomach.Chemotherapy consisting of folinic acid,fluorouracil,and oxaliplatin was administered for three cycles,and was changed to gemcitabine monotherapy because of adverse effects and increased serum tumor marker levels.The patient remained asymptomatic during a 12-month follow-up.CONCLUSION Pancreatic heterotopy should be considered as source of a potentially malignant lesion,and early treatment or close monitoring for aberrant pancreas is recommended.

Endoscopic submucosal dissection as alternative to surgery for complicated gastric heterotopic pancreas

BACKGROUND Gastric heterotopic pancreas(GHP)is generally asymptomatic and rarely features complications such as pancreatitis,pseudocysts,gastric outlet obstruction,bleeding,obstructive jaundice,or intussusception.However,the treatment of complicated GHP is challenging and often requires surgical resection.AIM To investigate the clinical outcomes of endoscopic submucosal dissection(ESD)as alternative to surgical resection for complicated GHP.METHODS This is a single-center,retrospective study.Between January 2013 and December 2017,a total of 5 patients underwent ESD for complicated GHP at Asan Medical Center.Patients who were diagnosed with complicated GHP were treated conservatively as with general practice for acute pancreatitis.After conservative management for resolving the acute phase of pancreatitis,ESD was performed as definitive treatment for complicated GHP.ESD was performed using the conventional method under conscious sedation.The clinical features of patients and tumors,procedure-related characteristics,and long-term outcomes were investigated.RESULTS The age of the 5 patients ranged from 28-43 years.Two of the patients were males.All lesions were located in the greater curvature of the antrum.On endoscopic ultrasonography during the pain episode,all lesions were located across the muscularis mucosa,submucosa,and proper muscle layers.The median lesion size was 20[interquartile range(IQR),18-35]during the pain episode at the time of the diagnosis of complicated GHP,and 15 mm(IQR,9-33)at the time of ESD after conservative treatment.The procedure time ranged from 15-120 min.There were no procedure-related adverse events such as perforation or bleeding.The length of hospital stay after the procedure ranged from 2-4 d.All patients were symptom free during the median follow-up period of 46.0 mo(IQR,39-60).CONCLUSION ESD appears to be a feasible and effective treatment option for complicated GHP based on the favorable clinical outcomes.

HER2-positive adenocarcinoma arising from heterotopic pancreas tissue in the duodenum:A case report

BACKGROUND Adenocarcinoma originating from heterotopic pancreas tissue is a rare disease.Furthermore,to our knowledge,no HER2-positive cases in the duodenum have been reported in the scientific literature nor has the efficacy of trastuzumab treatment for the disease been reported.CASE SUMMARY A 65-year-old woman whose clinical diagnosis was unresectable advanced duodenal cancer with HER2 overexpression responded well to trastuzumab chemotherapy.The main tumor in the duodenum reduced drastically.The patient underwent pancreaticoduodenectomy and lymph node dissection.A small number of cancer cells remained in the submucosal layer of the duodenum and pancreas head.After histological and immunohistochemical examination,the patient was diagnosed with duodenal adenocarcinoma originating from heterotopic pancreas tissue.CONCLUSION Trastuzumab treatment is effective in HER2-positive adenocarcinoma originating from heterotopic pancreas tissue in the duodenum.

Asymptomatic gastric adenomyoma and heterotopic pancreas in a patient with pancreatic cancer: A case report and review of the literature

BACKGROUND Gastric adenomyoma(GA)is a rare submucosal benign neoplasm that occurs mostly in the gastric antrum and is often misdiagnosed.No standard treatment has been established for this disease in cases of malignancy.CASE SUMMARY A 75-year-old woman with a 10-year history of hypertension was admitted to the Emergency Department of our hospital complaining of paroxysmal exacerbation of acute abdominal pain for 1 d with no apparent cause.Enhanced computed tomography and magnetic resonance imaging indicated a mass in the caudal pancreas,cholecystitis,and cholecystic polypus.Gastrointestinal endoscopy showed a mass arising from the gastric antrum.Due to the imaging findings,pancreatic cancer(PC),gastric lesion,cholecystitis,and cholecystic polypus were our primary consideration.Radical pancreatectomy,splenectomy,and cholecystectomy were performed successfully,and the gastric tumor was locally resected.Postoperative paraffin specimens confirmed the diagnosis of caudal PC,GA,and heterotopic pancreas(HP).Unfortunately,the patient died 13 mo later due to PC metastases to the liver,lung,and adrenal glands.CONCLUSION GA is a rare benign disease,especially when occurring with HP.It may stem from the same origin as HP.This is the first case report to date of a patient suffering from the simultaneous occurrence of GA,HP,and PC.GA is a lesion that can mimic other benign or malignant gastrointestinal diseases;thus,a definitive diagnosis depends on postoperative pathological biopsy.Although GA and HP are both benign lesions,they should be resected because there is a chance of malignancy.Additional research should be conducted to better understand these submucosal lesions.

Heterotopic pancreas: report of 7 patients

Objective: To discuss the clinical features of patients with heterotopic pancreas, their diagnosis and surgi- cal treatment. Methods: Seven patients with heterotopic pancreas were treated surgically in our hospital from August 1992 to March 1999. Results: Of the 7 patients, 4 had heterotopic pancre- as located in the duodenum, 2 in the jejunum, and 1 in the stomach. Four patients experienced abdominal pain, 3 icterus, 1 duodenal obstruction, and 1 diges- tive tract bleeding. Three patients were complicated by cholelithiasis, and 1 patient was complicated by diverticulum of the jejunum. All seven patients were misdiagnosed or undefined preoperatively. They un- derwent surgery and were confirmed pathologically. Conclusions: Heterotopic pancreas is extremely diffi- cult to diagnose before operation since no specific clinical signs are seen in such patients. Once diag- nosed with symptoms or not, the patients should un- dergo surgery for correct diagnosis and avoidance of relative complications.

Analysis of diagnosis and treatment of heterotopic pancreas

A case of heterotopic pancreas in the Third Affiliated Hospital of Inner Mongolia Medical University was recorded and analyzed on the basis of diagnosis,physical examination and treatment.Misdiagnosis of gastrointestinal stromal tumor(GIST)is very common since it is a rare disease.So this paper aims to enhance the doctors’awareness of GIST during clinical practice.

Ectopic pancreas at the ampulla of Vater diagnosed with endoscopic snare papillectomy: A case report and review of literature

BACKGROUND Ectopic pancreas is a rare developmental anomaly that results in a variety of clinical presentations.Patients with ectopic pancreas are mostly asymptomatic,and if symptomatic,symptoms are usually nonspecific and determined by the location of the lesion and the various complications arising from it.Ectopic pancreas at the ampulla of Vater(EPAV)is rare and typically diagnosed after highly morbid surgical procedures such as pancreaticoduodenectomy or ampullectomy.To our knowledge,we report the first case of confirmed EPAV with a minimally invasive intervention.CASE SUMMARY A 71-year-old male with coronary artery disease,presented to us with new-onset dyspepsia with imaging studies revealing a‘double duct sign’secondary to a small subepithelial ampullary lesion.His hematological and biochemical investigations were normal.His age,comorbidity,poor diagnostic accuracy of endoscopy,biopsies and imaging techniques for subepithelial ampullary lesions,and suspicion of malignancy made us acquire histological diagnosis before morbid surgical intervention.We performed balloon-catheter-assisted endoscopic snare papillectomy which aided us to achieve en bloc resection of the ampulla for histopathological diagnosis and staging.The patient’s post-procedure recovery was uneventful.The en bloc resected specimen revealed ectopic pancreatic tissue in the ampullary region.Thus,the benign histopathology avoided morbid surgical intervention in our patient.At 15 mo follow-up,the patient is asymptomatic.CONCLUSION EPAV is rare and remains challenging to diagnose.This rare entity should be included in the differential diagnosis of subepithelial ampullary lesions.Endoscopic en bloc resection of the papilla may play a vital role as a diagnostic and therapeutic option for preoperative histological diagnosis and staging to avoid morbid surgical procedures.

Duodenal heterotopic pancreatic tissue:a case report and literature review

Heterotopic pancreas is an aberration in the developmental profile of the pancreas.This entity is rarely symptomatic and its malignant transformation is even rarer.However,when present,it poses a diagnostic dilemma to clinicians,as little help comes from gastroenteroscopy and imaging.Surgical exploration remains the only option at times,and it is the histopathological examination that finally clears the mist.This case report reveals the elusive nature of malignancy in heterotopic pancreas in the duodenum.