Heterotopic Pancreas: Unusual Cause of Usual Diarrhoea.

Heterotopic pancreas (HP) also known as ectopic pancreas, pancreatic crest or accessory pancreas is the normal pancreatic tissue, found in a remote area other than its natural location, with no anatomic or vascular connection to main pancreatic tissue. It is a rare congenital anomaly and has been reported at many locations such as stomach (antrum) and small intestine. HP is usually an incidental finding and asymptomatic, however there are reports of pancreatitis, obstruction, perforation and malignant transformation as uncommon manifestations. Diagnosis of HP is primarily based on histological examination either by biopsy or surgical excision. Surgery is the standard treatment for symptomatic HP patients. Herein, we present a case of a 58-year-old female, who presented to us with intractable diarrhoea due to HP in the jejunum and underwent minimally invasive surgery for definitive diagnosis and treatment.

Jejunal Ectopic Pancreatic Tissue Rest as Lead Point in Patients with Intussusception: A Rare Case Report and Review of Literature.

Background: Ectopic pancreas (EP), characterized by pancreatic tissue outside its usual location, poses diagnostic challenges due to its asymptomatic nature in most cases. Gastric lesions are often symptomatic, causing epigastric pain or gastric outlet obstruction. Rarely, jejunal lesions can lead to intestinal obstruction or intussusception. The elusive preoperative diagnosis lacks specific biochemical markers, relying on intraoperative biopsy and histopathology as gold standards. Case Presentation: We present a unique case of a 37-year-old female with 12-hour crampy abdominal pain, bilious vomiting, blood-mixed diarrhea, abdominal distension, and obstructive symptoms. Imaging revealed jejunojejunal intussusception with a jejunal mass as the lead point. Intraoperatively, also identified jejunal mass as lead point. Resection of mass and end-to-end jejunojejunal anastomosis were performed, resulting in a successful outcome. Histopathological examination identified a type I ectopic pancreas within the muscularis propria. Twelve months postoperatively, the patient exhibited no stricture or recurrence. Conclusion: Ectopic pancreas, with varied symptoms, poses diagnostic hurdles. Despite the diagnostic challenges, surgical excision remains the optimal treatment for symptomatic cases. This report contributes to the limited literature on ectopic pancreas, emphasizing the importance of considering this entity in the differential diagnosis of gastrointestinal pathology.

Pancreatic heterotopia of the gallbladder - possible cause of right upper quadrant pain, a case report.

Pancreatic heterotopia is characterized by ectopic pancreatic tissue found outside the pancreas without any anatomical or vascular connection to the pancreas. Pancreatic heterotopia of the gallbladder is a rare histological finding; there have been only a handful of cases described in the literature. Pancreatic heterotopia of the gallbladder is usually diagnosed incidentally at histological examination following cholecystectomy or autopsy. Clinical presentation of pancreatic heterotopia of the gallbladder can vary from biliary colic, biliary obstruction or it can be completely asymptomatic. It has been suggested that gallbladder pancreatic heterotopia may lead to pancreatitis of this ectopic tissue, which may present differently to typical biliary colic. Here, we present the case of a 43-year-old male that presented with 2 years of significant postprandial nausea and right upper quadrant pain. Histopathology following cholecystectomy revealed chronic cholecystitis with cholelithiasis, in addition to a focus of pancreatic heterotopia in the gallbladder wall.

Pancreatic adenocarcinoma arising from an ectopic pancreas in a cat.

An 8-year-old male neutered Korean shorthair cat presented with chronic vomiting. Radiographically, an oval-shaped soft tissue abdominal mass caudoventral to the left kidney was detected. On ultrasonography, the hypoechoic mass was well-defined with thick, irregular, and hyperechoic margins and had no continuity with the pancreas or other adjacent organs. The mass was surgically excised. Areas of atypical pancreatic acinar epithelial cells were identified histopathologically. Postoperative CT demonstrated a normal pancreas in the expected anatomical region. Based on diagnostic imaging, surgical and histopathology findings, the mass was diagnosed as a well-differentiated pancreatic acinar cell adenocarcinoma arising from ectopic pancreatic tissue.

Pancreatic Heterotopia at the Gastroesophageal Junction: A Case Report and Review of the Literature.

Pancreatic heterotopia is characterized by the presence of pancreatic tissue in a location outside of its typical anatomical position. Symptoms of pancreatic heterotopia vary based on the location of the ectopic tissue. It is commonly asymptomatic and often diagnosed incidentally during routine endoscopy. Clinically significant pancreatic heterotopia is often secondary to inflammation, bleeding, obstruction, and malignant transformation. The most common location of heterotopic pancreas is within 5 cm of the pylorus usually on the greater curvature. Involvement of the gastroesophageal junction is extremely rare. In this report, we describe the case of a 57-year-old woman who was diagnosed with ectopic pancreatic tissue at the gastroesophageal junction by esophagogastroduodenoscopy after presenting with symptoms of dyspepsia.

A case of ectopic pancreas of the stomach accompanied by intraductal papillary mucinous neoplasm with GNAS mutation.

BACKGROUND: Ectopic pancreas is basically a benign disease and is not always necessary to be removed. However, all types of neoplasms occurring in the normal pancreas such as ductal adenocarcinomas and intraductal papillary mucinous neoplasms (IPMNs) may develop even within ectopic pancreas. We recently encountered an extremely rare case of ectopic pancreas in the gastric antrum associated with IPMN possessing a GNAS mutation. CASE PRESENTATION: A 71-year-old Japanese woman complained of epigastric pain. Computed tomography and upper gastrointestinal endoscopy showed an intramural cystic mass in the antrum of the stomach. Endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) biopsy did not give a definitive diagnosis, and the patient underwent resection of the lesion. Histology of the resected specimen showed that the gastric intramural lesion was ectopic pancreas. Moreover, the lesion contained dilated duct components with tubulo-villous epithelial proliferation consistent with pancreatic IPMN. Since the covering epithelial cells had highly atypical nuclei, the lesion was diagnosed as IPMN with high grade dysplasia. Immunohistochemistry showed that the IPMN component showed to be MUC2-, MUC5AC-, and CDX2-positive but MUC1- and MUC6-negative. Mutational analyses using genomic DNA revealed that the IPMN component had a mutation of GNAS at exon 8 (Arg201Cys). CONCLUSION: We finally diagnosed this case as gastric ectopic pancreas accompanied by intestinal type IPMN with high grade dysplasia possessing GNAS mutation. Although there were 17 cases of ectopic pancreas with IPMN including 6 cases of gastric ones reported in the English literature, this is the first case of ectopic pancreas with IPMN which was proved to have GNAS mutation. Intimate preoperative examinations including imaging analyses and EUS-FNA biopsy/cytology are recommended to decide whether the lesion has to be resected or not even if they are not effective for getting the right diagnosis.

Jejunal Intussusception Due to Heterotopic Pancreas: A Case Report.

Intussusception in adults is rare. Even more unusual is jejunal intussusception secondary to a heterotopic pancreas. The presence of pancreatic tissue in an ectopic location and lacking contiguity with the main pancreatic gland is defined as pancreatic heterotopia. It is very rarely symptomatic and usually diagnosed incidentally during surgical intervention for other conditions. We report the case of a 78-year-old lady who presented with a history of constipation, abdominal pain, and vomiting. A CT scan revealed features of a proximal jejunojejunal intussusception secondary to a small soft tissue density lead point. After laparotomy and segmental jejunal resection, histopathology confirmed the diagnosis of ectopic pancreatic tissue as the lead point. Although uncommon, heterotopic pancreatic tissue should be included in the differential diagnosis for proximal small bowel intussusception.

Unusual Tissue - Unusual Issue: Pancreatic Heterotopia Presenting as Gastric Outlet Obstruction.

Pancreatic heterotopia (PH) is a common, but typically small (<1 cm), incidental and asymptomatic finding; however, PH should be considered even for large and symptomatic upper gastrointestinal masses. A 27-year-old white woman presented with a 3-week history of burning epigastric pain, nausea, early satiety, and constipation. Physical examination revealed epigastric and right upper quadrant tenderness with normal laboratory workup, but imaging revealed a 5-cm, partly cystic mass arising from the gastric antrum with resulting pyloric stenosis and partial gastric outlet obstruction. Endoscopic ultrasound-guided fine needle aspiration revealed PH - an anomalous pancreatic tissue lying in a nonphysiological site. The patient ultimately underwent a resection and recovered uneventfully, with a complete pathologic examination revealing normal exocrine pancreatic tissue (PH type 2) without malignant transformation. We report a case of heterotopic pancreas manifesting as severe gastric outlet obstruction, in addition to a thorough diagnostic workup and surgical follow-up, in a young adult. Differential diagnoses and features that speak to benignity of a large, symptomatic mass lesion (PH in particular) are discussed.

Pancreatic Endocrine Heterotopia Involving Meckel's Diverticulum: A Potential Mimic of Neuroendocrine Tumor.

Pancreatic heterotopia most commonly occurs in the upper gastrointestinal tract, but can occur in other sites, including Meckel's diverticulum. When multiple histologic elements of the pancreatic tissue (acini, ducts, and endocrine cells) are present, the diagnosis is typically straightforward. In this article, we report a rare case of pure endocrine pancreatic heterotopia involving a Meckel's diverticulum, a potential mimic of a well-differentiated neuroendocrine tumor. Several features were useful in making the distinction, including lack of desmoplasia and mass forming lesion, and immunohistochemical staining in a physiological pattern similar to that of islets of Langerhans. It is important for pathologists to be aware of this entity and its features to avoid misdiagnosis of a neuroendocrine tumor.

Pancreatic heterotopia in mesentery.

INTRODUCTION: The pancreatic heterotopia of fortuitous operational discovery remains rare because of the medical imagery progress. We report a mesenteric localisation of aberrant pancreas identified during the assumption of responsibility of an abdominal emergency. OBSERVATION: A 26-year-old man was allowed with the urgencies for an acute obstruction of the small bowel. Surgical exploration revealed a distal support an ileo-parietal adherence near to an inflammatory hearth of appendicular origin. We noted, in addition to the mesentery of the first jejunal loop, a bilobate mass of glandular-like tissue with a pancreatic aspect. The adjacent jejunal handles were macroscopically healthy. The Pathological examination of the mass confirmed the existence of a mixed glandular exocrine and endocrine origin of pancreatic tissue, type I of the Heinrich's classification. CONCLUSION: This rare topographic entity of fortuitous discovery underlines the need for a meticulous surgical exploration of the abdominal cavity particularly in urgency when a summarized radiological assessment does not permit a complete morphological study.

Loss of GATA4 causes ectopic pancreas in the stomach.

Pancreatic heterotopia is defined as pancreatic tissue outside its normal location in the body and anatomically separated from the pancreas. In this work we have analyzed the stomach glandular epithelium of Gata4 flox/flox ; Pdx1-Cre mice (Gata4KO mice). We found that Gata4KO glandular epithelium displays an atypical morphology similar to the cornified squamous epithelium and exhibits upregulation of forestomach markers. The developing gastric units fail to form properly, and the glandular epithelial cells do not express markers of gastric gland in the absence of GATA4. Of interest, the developing glands of the Gata4KO stomach express pancreatic cell markers. Furthermore, a mass of pancreatic tissue located in the subserosa of the Gata4KO stomach is observed at adult stages. Heterotopic pancreas found in Gata4-deficient mice contains all three pancreatic cell lineages: ductal, acinar, and endocrine. Moreover, Gata4 expression is downregulated in ectopic pancreatic tissue of some human biopsy samples. © 2019 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

Pancreatic heterotopia in a neonatal abdominopelvic cyst.

Pancreatic heterotopia is a rare congenital anomaly more commonly incidentally found in adults. While intra-abdominal cysts found on prenatal imaging are not uncommon, we examine a case of pancreatic heterotopia presenting in an abdominopelvic cyst on prenatal imaging. At birth, the neonate was found to have hypoglycemia unresponsive to treatment that resolved after cyst resection. We will review the etiology, imaging findings and clinical course of this anomaly, which should be considered in the differential diagnosis of newborns presenting with unexplained hypoglycemia.

Pancreatic and gastric heterotopic tissue presenting as a symptomatic gallbladder mass: A case report and literature review.

Pancreatic and gastric heterotopia is an uncommon congenital anomaly in which gastric and/or pancreatic tissue is found outside of its anatomic location. In the majority of patients, lesions are found incidentally because they tend to be asymptomatic. However, lesions may become symptomatic when inflammation, obstruction, and bleeding occurs. Depending on tissue size and location they can harvest a landscape of nonspecific symptoms, causing a delay in diagnosis. Heterotopic tissue of either gastric or pancreatic origin have been reported in literature, however the presence of combined gastric and pancreatic heterotopic tissue is exceedingly rare. We report a case of an 18-year-old female with polypoid gastric and pancreatic heterotopia and focal intestinal metaplasia of the gallbladder with clinical findings of acute cholecystitis. In this literature review, we synthesize the clinical significance, histopathological features, diagnosis and management of pancreatic and gastric heterotopic tissue.

Ectopic pancreas in the upper gastrointestinal tract: Is endosonographic diagnosis reliable? Data from the German Endoscopic Ultrasound Registry and review of the literature.

BACKGROUND: Ectopic pancreas (EP) belongs to the most frequent subepithelial lesions (SELs) of the upper gastrointestinal (GI) tract. In the majority of cases, it is detected incidentally. Differential diagnosis from mesenchymal subepithelial tumors may be difficult. METHODS: Among 24,308 endosonographic examinations and interventions, which were prospectively enrolled in the database of the German Endoscopic Ultrasound (EUS) Registry from January 2009 to August 2013, 575 were performed for suspected SELs of the upper GI tract. Sixty three cases of EP of the upper GI tract (stomach, n = 53; duodenum, n = 10; esophagus, n = 0) were extracted and retrospectively reviewed. RESULTS: In 65.1% of cases, radial echoendoscopes or radial miniprobes were used for examination. Nearly 84% of EP was found in the stomach, 16% in the duodenum, none in the esophagus. In 88.9% of cases, the EUS examination discerned the layer of origin. In 59% of cases EP was described as a heterogeneous, in 28.6% as a homogeneous-hypoechoic and in 7.9% as a homogeneous-echogenic subepithelial mass lesion. Mean diameter was 13.0 mm × 8.1 mm, the mean ratio between long and short axis diameter was 1.75. EUS-guided fine needle aspiration (EUS-FNA) was used to accomplish cytological or histological diagnosis in only 6.3% of cases. CONCLUSIONS: EP accounts for 11% of all EUS examinations performed for subepithelial lesions of the upper GI tract and prospectively enrolled in the German EUS registry. Rather than being an eyecatcher, EP is a chameleon with numerous differential diagnoses. In selected cases, EUS-FNA may help clarifying the diagnosis.

Clinical impact of duodenal pancreatic heterotopia - Is there a need for surgical treatment?

BACKGROUND: Pancreatic heterotopia (PH) is defined as ectopic pancreatic tissue outside the normal pancreas and its vasculature and duct system. Most frequently, PH is detected incidentally by histopathological examination. The aim of the present study was to analyze a large single-center series of duodenal PH with respect to the clinical presentation. METHODS: A prospective pancreatic database was retrospectively analyzed for cases of PH of the duodenum. All pancreatic and duodenal resections performed between January 2000 and October 2015 were included and screened for histopathologically proven duodenal PH. PH was classified according to Heinrich's classification (Type I acini, ducts, and islet cells; Type II acini and ducts; Type III only ducts). RESULTS: A total of 1274 pancreatic and duodenal resections were performed within the study period, and 67 cases of PH (5.3%) were identified. The respective patients were predominantly male (72%) and either underwent pancreatoduodenectomy (n = 60); a limited pancreas resection with partial duodenal resection (n = 4); distal pancreatectomy with partial duodenal resection (n = 1); total pancreatectomy (n = 1); or enucleation (n = 1). Whereas 65 patients (83.5%) were asymptomatic, 11 patients (18.4%) presented with symptoms related to PH (most frequently with abdominal pain [72%] and duodenal obstruction [55%]). Of those, seven patients (63.6%) had chronic pancreatitis in the heterotopic pancreas. The risk of malignant transformation into adenocarcinoma was 2.9%. CONCLUSIONS: PH is found in approximately 5% of pancreatic or duodenal resections and is generally asymptomatic. Chronic pancreatitis is not uncommon in heterotopic pancreatic tissue, and even there is a risk of malignant transformation. PH should be considered for the differential diagnosis of duodenal lesions and surgery should be considered, especially in symptomatic cases.

Cyst of the gastric wall arising from heterotopic pancreas: report of a case.

Heterotopia of pancreatic tissue is a common developmental anomaly, affecting predominantly the gastrointestinal tract. The case of a symptomatic cyst arising from the posterior gastric wall in a 40-year-old man is presented, undergoing laparoscopic gastric wedge resection. Pathology report described a cyst of the gastric wall lined by ductal pancreatic epithelium.

Pediatric Meckel's Diverticulum: Report of 208 Cases and Review of the Literature.

We report a review of 208 cases of Meckel's diverticulum among pediatric patients from one single institution. One of the aims of this report is to highlight the different diagnostic modalities of Meckel's diverticulum since a majority of cases is undiagnosed prior to surgery. Our review shows 58 cases containing gastric and/or pancreatic heterotopic tissue, including two unique cases. The first case reported is a desmoid tumor arising at the tip of diverticulum, a case that, to our knowledge, has not been previously reported. The second case involves a female patient appearing with an acute abdomen thought to be appendicitis, instead surgery revealed a diverticulum arising from the ileum. The cause of acute abdomen was due to gonococcal infection. In conclusion, we hope that this large series of Meckel's cases will enrich our readers on the differential diagnosis and preoperative diagnostic techniques of Meckels' diverticulum.

Pancreatic and Gastric Heterotopia with Associated Submucosal Lipoma Presenting as a 7-cm Obstructive Tumor of the Ileum: Resection with Double Balloon Enteroscopy.

Pancreatic and gastric heterotopias are rare clinical entities which have been identified throughout the entire length of the gastrointestinal tract. Combined gastric and pancreatic heterotopias, although unusual, have been described in the duodenum and jejunum, and in other structures, including Meckel's diverticulum and the ampulla of Vater. We report a novel case of pancreatic and gastric heterotopia with an associated submucosal lipoma in a 38-year-old female with a recent history of rectal cancer and chronic crampy abdominal pain. On computed tomography, a 7-cm luminal polypoid mass extending into the distal ileum was discovered. The mass was successfully resected using retrograde double balloon enteroscopy. We believe this is the first report of all three histological entities co-existing in an obstructive ileal lesion in an adult. It highlights endoscopic resection trough double enteroscopy as a safe alternative to more invasive surgical approaches for this type of lesion.

Heterotopia pancreática yeyuna yuxtahepática y en quiste de conducto colédoco / Jejunal juxtahepatic and common bile duct (ductus choledochus) cystic pancreatic heterotopia

Se exponen 3 casos con hallazgo incidental de páncreas heterotópico, en autopsia y 2 piezas quirúrgicas para hacer una breve revisión del tema. Casos: 1. Mujer de 53 años de edad fallecida por neumonía de focos múltiples. Durante el estudio post mortem se encontró, a nivel del segmento yeyunal, un nódulo constituido histológicamente por múltiples conductos con epitelio columnar y fibras anchas desorganizadas de músculo liso. 2. Preescolar varón de 2 años 11 meses de edad con diagnóstico de quiste de colédoco y resección del mismo. En uno de los cortes de pared se observó una banda de tejido que a la microscopía de luz correspondía a tejido pancreático sin alteraciones. 3. Escolar mujer de 6 años 10 meses de edad con diagnóstico de síndrome de Byler candidata a transplante hepático. Los cortes histológicos del explante en la región del hilio revelaron grupos multifocales de conductos y acinos pancreáticos sin presencia de islotes. Conclusión: La heterotopia pancreática es un hallazgo infrecuente que se puede observar a cualquier nivel del tracto gastrointestinal e inclusive fuera del mismo, por lo que la caracterización histopatológica de esta alteración permite distinguirla de otras lesiones. Pese a su conducta habitualmente benigna y asintomática, ocasionalmente puede dar origen a cuadros obstructivos, hemorrágicos, inflamatorios o neoplásicos.

We report three cases of pancreatic heterotopia incidentally found (one in autopsy and two in surgical pieces) with a brief review of the literature. Cases: 1. A fifty-three-year-old woman who died of bronchopneumonia. During post-mortem examination, a nodule (hystologically formed by multiple ducts lined by columnar epithelium and broad disarranged smooth muscle fibers) was found at the level of jejune. 2. 5-year, 11-month-old male with diagnosis ofcholedochal cyst. In the resected specimen, one of the mural slices showed a tissue stripe that under light-microscope examination corresponded to normal pancreatic tissue. 3. 6-year, 10-month-old female diagnosed with Byler syndrome who was recipient of liver transplant. Slices taken from the hilum in the resected specimen revealed multiple clusters of pancreatic acini and ducts without evidence of endocrine islets. Conclusion: Pancreatic heterotopia is an uncommon finding, which may be found at any level of the gastrointestinal tract, and even outside it. Histopathologic studies allow to distinguish this disorder from other lesions. Despite its commonly benign and asymptomatic behaviour, it may sometimes produce obstruction, hemorrhage, inflammation or neoplasms.

Ectopic pancreas: a diagnostic dilemma.

An ectopic pancreas is defined as pancreatic tissue lacking vascular or anatomic communication with the normal body of the pancreas. It is rarely symptomatic as it is found incidentally at laparotomy most of the time. Despite advances in diagnostic modalities, it still remains a challenge to the clinician to differentiate it from a neoplasm. It is prudent to differentiate it from neoplastic etiologies, as simple surgical excision can potentially be curative. We discuss the presentation, diagnosis, and treatment of an interesting case of ectopic pancreas presenting as a gastric antral tumor.