Pancreatic Heterotopia Research Articles

Su1305 A SAFE AND COST EFFECTIVE STRATEGY FOR THE MANAGEMENT OF SMALL ASYMPTOMATIC GASTRIC SUBEPITHELIAL TUMORS: A CASE SERIES

Gastric subepithelial tumors (SETs) are relatively common incidental findings encountered on endoscopy. Current ASGE guidelines for small SETs (<2cm) arising from the second and third echolayers recommend tissue acquisition for diagnosis or endoscopic mucosal resection (EMR) for lesions that are symptomatic or increase in size over time. If tissue acquisition is non-diagnostic, it is recommended that these patients have endoscopic ultrasound (EUS) surveillance at poorly defined intervals. In addition to the cumulative risk of anesthesia and significant cost of repeated endoscopy, diagnosis may be challenging because of the low diagnostic yield of tissue acquisition methods currently available for these SETs and the limited accuracy of EUS with endosonographic imaging alone. We propose that routine EMR of asymptomatic gastric SETs <2cm arising from the second and third echolayers is a safe and more cost effective strategy for management than current surveillance recommendations. Provation was queried to identify patients undergoing EUS with a coded diagnosis of “gastric nodule” or “intramural lesion” from September 2017 through September 2019 at our institution. Inclusion criteria for analysis were gastric SETs <2cm arising from the second and third echolayers and removed with EMR. These cases were reviewed for EMR-related complications and pathology of removed specimens. Of the 96 cases identified, 13 patients with 14 distinct lesions met inclusion criteria. All lesions were removed via endoscopic cap band mucosectomy. The mean age of patients was 58.6 years with a female predominance (69.2%). The mean size of these gastric SETs was 7.4 mm. Lesions were most commonly located in the gastric antrum (64%). Pancreatic heterotopia (29%), gastric mucosa (29%), and inflammatory fibroid polyps (23%) were most the most commonly reported pathologic findings. Notably, no carcinoid or other neoplastic lesions were identified. Hemostatic clips were used in 50% of removed lesions. No procedural complications such as perforation were identified. Currently available tissue acquisition methods for small gastric SETs are not always diagnostic, obligating many patients to the EUS surveillance pathway. This results in repeated procedures with a cumulative risk of adverse events. Our results are consistent with published literature showing that asymptomatic SETs <2 cm originating from the second and third echolayers are often benign and can be safely removed by EMR. The more universal use of EMR in this context would increase diagnostic yield, reduce procedural risks, and provide substantial cost savings by eliminating the need for surveillance. As evidence mounts for the safe and efficacious use of EMR in this context, updated guidelines to include this as a viable option need to be considered.

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.

Mucinous cystadenoma arising from a pancreatic heterotopia.

Mucinous pancreatic cystadenoma (MCA) is a premalignant lesion usually located in the body and tail of the pancreas, characteristically in females in their fifties and sixties. The definitive diagnosis relies on the presence of columnar mucin-producing epithelium and ovarian-type stroma. MCA arising from a pancreatic heterotopia (PH) has been rarely reported.

Intrahepatic ectopic pancreas

Ectopic pancreas (or aberrant or pancreatic heterotopia) is defined as pancreatic tissue outside the boundaries of the pancreas, without anatomic connection to the main gland. Its incidence is between 0.5% and 14% in autopsy series, but it is probably underestimated because it is often asymptomatic. It most commonly affects the duodenum (30-35%), the stomach (30%), and the jejunum (15%). Hepatic involvement is exceptional. We here report a new case of unexpected detection. The study involved a 54-year-old, hypertense female patient who had undergone surgery for adenocarcinoma of the sigmoid classified pT3N1b (A) two years before. Disease progression was marked by the occurrence of multiple predominant secondary nodules in the right hepatic lobe (B). Neoadjuvant chemotherapy (4 cicles of FOLFOX-ERBITUX) was started. The patient received right lobectomy. The histological examination of the different surgical specimens confirmed the presence of secondary, moderately differentiated hepatic adenocarcinoma of colorectal origin with healthy surgical excision margins. The surrounding liver was the site of unsystematized macrovacuolar steatosis in 20% of cases. It was associated with a focus of pancreatic heterotopia, including some acini and excretory canals arranged in lobules (C, D). The postoperative course was simple; however, patient’s outcome was marked by recurrence of cancer in the left lobe of the liver.

Heterotopia pancreática en pólipo gástrico. Presentación de Caso

La heterotopia pancreática es un hallazgo poco frecuente, en ocasiones accidental, que se puede observar en cualquier nivel del tracto digestivo e inclusive fuera del mismo; es congénita y su patogenia está en discusión. Aunque su forma de presentación es lenta y progresiva y su comportamiento benigno y asintomático, en ocasiones puede originar manifestaciones obstructivas, hemorrágicas, inflamatorias y neoplásicas. En este último caso es imprescindible el diagnóstico histopatológico. Se presenta el caso de un paciente sintomático con heterotopia pancreática a nivel del píloro gástrico cuya localización es bastante infrecuente.

1761 Heterotopic Pancreatic, Sebaceous, and Gastric Tissue: Report of Case and Review of the Literature

INTRODUCTION: Heterotopic mucosa refers to the presence of normal tissue at a foreign site, and is thought to be either congenital from an error during organogenesis or metaplastic from an error during differentiation of pluripotent cells. We present a rare case of pancreatic and sebaceous gland heterotopia in the esophagus and gastric mucosal heterotopia in the rectum. To our knowledge, this is the first case with three types of heterotopia found in a single patient. CASE DESCRIPTION/METHODS: A 61-year-old man with GERD presented with periodic dysphagia to solids. He denied regurgitation, heartburn, melena, hematochezia, constipation, and diarrhea. Physical exam was unremarkable. Barium swallow showed a small hiatal hernia, and mild concentric narrowing at the GE junction. EGD showed a low grade Schatzki ring and diffuse beige 2-3 mm specks in middle third of esophagus. A colonoscopy was also scheduled for screening purposes and showed a 20 mm semi-sessile polyp in the rectum. Biopsies identified focal pancreatic and sebaceous gland heterotopia in the esophagus and gastric fundus heterotopia in the rectum. DISCUSSION: Heterotopic pancreatic, sebaceous, and gastric mucosa have been described along the entire GI tract. Our literature review identified 11 case reports of pancreatic heterotopia in the esophagus, 23 case reports of heterotopic sebaceous glands in the esophagus, and 73 case reports of gastric heterotopia in the rectum. However, no case reports found a single patient with all three types of heterotopia. In all three types, there was a male predominance, and ages ranged from birth to 6th decade of life. The origin of the heterotopic tissues is hypothesized to be either congenital vs metaplastic. As the combination of organ heterotopia described here includes components of mesoderm and endoderm, there was presumably incomplete cellular differentiation in weeks 2-3 of embryonic development. Patients can be asymptomatic or present with dysphagia, epigastric pain, or reflux with heterotopic pancreatic mucosa and sebaceous glands in the esophagus, and hematochezia with heterotopic gastric mucosa in the rectum. Treatment for the heterotopic tissues vary but surgical or endoscopic resection is typically pursued. Given that the tissue is often removed, it is unclear the malignant potential of the lesions and close follow up is necessary. Ultimately, presentations such as hematochezia and dysphagia may have rare etiologies and necessitates sufficient biopsies.

1704 A Case of Heterotopic Pancreas Discovered in the Distal Esophagus

INTRODUCTION: Heterotopic pancreas (HP) is a congenital abnormality that represents ectopic pancreatic tissue that does not have anatomic, vascular or ductal continuity. The incidence is hard to determine, however, the prevalence of HP is 0.55% to 13.7% on autopsy, 0.2% to 0.5% of abdominal operations, and 0.9% of gastrectomies. HP is most commonly found in the stomach, duodenum, proximal jejunum and rarely, in the esophagus. Approximately 15 cases have been reported in the literature of HP located in the esophagus. CASE DESCRIPTION/METHODS: We present a case of a 70-year-old male with a past medical history significant for hypertension, hypercholesterolemia, melanoma, and atrial fibrillation who underwent an esophagogastroduodenoscopy (EGD) for evaluation of heartburn and nausea. Incidentally a 5 mm polypoid appearing lesion in the distal esophagus above the gastroesophageal junction was found. Biopsy results are usually nondiagnostic, however, in our patient, histology revealed squamous mucosa with active esophagitis and associated cardiac-type mucosa with focal pancreatic heterotopia and chronic inflammation in the distal esophagus. DISCUSSION: Complications may result from undiagnosed cases of HP including benign or malignant transformation of tissue, pseudocyst formation, pancreatitis, gastrointestinal bleeding, ulceration, gastric outlet obstruction, or intussusception. Treatment depends on the symptom and location. In asymptomatic patients, simple observation may be sufficient, however in patients with symptoms or complications, surgical or Ivor-Lewis Esophagectomy, or laparoscopic resection is recommended. In our case, the patient’s symptoms were treated conservatively with a proton pump inhibitor successfully.

Heterotrophic Pancreatic Rest Mimicking Tumor in Stomach: A case Report and Literature Review

Pancreatic heterotopia is an entity defined by extra pancreatic tissue without any anatomic or vascular continuity with the pancreas.

Image Case : Pancreatic Rest

18 years old female patient presented by epigastric pain and dyspepsia.Upper gastrointestinal endoscopy revealed umplicated small antral mass(pancreatic rest). Pancreatic rests are benign congenital anomalies defined as pancreatic tissue that lacks anatomic or vascular continuity with the pancreas itself . Also referred to as pancreatic heterotopia, heterotopic pancreas, ectopic pancreas, aberrant pancreas, and accessory pancreas. Although they can be located in many sites throughout the body, they are most commonly found in the upper gastrointestinal tract, with the gastric antrum being the most common site. The differential diagnosis of gastric antral subepithelial lesions includes benign entities such as pancreatic rests, lipomas, inflammatory polyps, hyperplastic mucosa, Leiomyoma and cysts as well as entities with malignant potential such as carcinoid tumors and gastrointestinal stromal tumors. Mucosal biopsies can exclude epithelial tumors, and EUS can help exclude other lesions (such as cysts, lipomas, stromal tumors, or extrinsic lesions). The treatment of symptomatic pancreatic rest is:Endoscopic band ligation snare polypectomy(EBLSP) or Surgery. EUS should be performed prior to endoscopic mucosal resection to ensure safety of resection by making sure the lesion is superficial, not vascular, and does not involve the muscularis propria.

Malignant Transformation of Ectopic Pancreas.

Malignant transformation of ectopic pancreas tissue is a diagnostic challenge as clinical symptoms and radiographic features of these tumors are non-specific. Given the rarity of these lesions, it is usually neither suspected nor included in the diagnostic workup of different tumors. We conducted a comprehensive literature review regarding malignancy arising from ectopic pancreas for a better understanding of its frequency, clinicopathological features, and prognosis. A literature search was performed in three major databases: PubMed, Cochrane, and Web of Science. Fifty-four well-documented cases of malignant ectopic pancreas were identified in the published literature. Our analysis provided the following observations: (1) there was a slight predominance of males over females; (2) most patients with malignant transformation of ectopic pancreas were middle-aged; (3) most commonly, the tumor was located in the stomach; (4) most tumors were adenocarcinomas; (5) most frequently, the malignancy arose within a type I heterotopia according to Heinrich classification; (6) macroscopically, a subepithelial-like appearance was most frequently observed; and (7) improved prognosis for ectopic pancreatic malignancies in comparison with reported survival data for orthotopic pancreatic cancer. Even if the majority of cases of ectopic pancreas are incidental findings and malignant transformation is a rare event, pancreatic heterotopy should be considered as a source of potentially malignant lesions.

Pancreatic-Type Mixed Acinar Neuroendocrine Carcinoma Arising in the Common Bile Duct: A Case Report

Abstract Mixed acinar neuroendocrine carcinoma (MAcNEC) of pancreatic type arising in an extrapancreatic location is extremely rare. We present a case of a 70-year-old woman with constipation, abdominal discomfort, and jaundice. Imaging studies revealed a 1.8-cm solid mass in the common bile duct causing dilatation of intrahepatic and extrahepatic bile ducts, which was resected. Microscopically, the tumor was limited to the wall of the bile duct, without involvement of the pancreas, and was composed of solid sheets and nests of relatively monomorphic cells with minimal to moderate amounts of cytoplasm, granular chromatin, focally prominent nucleoli, and up to 160 mitoses per 10 high-power fields. Immunohistochemistry showed equal to or greater than 30% positivity for synaptophysin, chromogranin, chymotrypsin, and trypsin. A diagnosis of MAcNEC was rendered. Immunohistochemistry plays a pivotal role in the identification of these tumors and discrimination from other related entities, neuroendocrine neoplasms in particular. Because pancreatic heterotopia has been described in the common bile duct, we postulate that this case of MAcNEC in the common bile duct, the first to be reported in the literature, may have arisen via malignant transformation of pancreatic heterotopia.

Esophageal Pancreatic Heterotopia and Dysphagia in the Absence of a Mass

Esophageal Pancreatic Heterotopia and Dysphagia in the Absence of a Mass

A Unique Presentation of Jejunal Submucosal Pancreatic Heterotopia as Overt Obscure Gastrointestinal Bleeding

A Unique Presentation of Jejunal Submucosal Pancreatic Heterotopia as Overt Obscure Gastrointestinal Bleeding

Heterotopic tissue in the gastrointestinal tract

Heterotopia of the gastrointestinal tract is acommon finding. This is due to the complex embryogenesis and the relative ease to detect heterotopic tissue during endoscopy. The reason for biopsy is mostly to rule out neoplasms or to define specific causes of inflammation. Heterotopic tissue can occur in any location of the gastrointestinal tract. The most frequent are gastric heterotopia, pancreatic heterotopia, and heterotopia of Brunner's gland. On rare occasions, heterotopic tissue of salivary gland type as well as heterotopias of apocrine glands, thyroid, and prostatic tissue have been described. The most frequently involved organs are the small intestine, in particular the duodenum, the esophagus, and the stomach. Heterotopia of the large bowel occurs exclusively in the rectum. Most heterotopias do not cause symptoms and are easily diagnosed by biopsy and histology. However, depending on location, size, and the kind of underlying heterotopic tissue, they may cause significant complications, such as inflammation, ulceration and perforation, obstruction, intussusception, and severe life-threatening bleeding. Another rare but significant complication is neoplasia. Gastric heterotopias may give rise to pyloric gland adenomas within the bowel or rarely adenocarcinomas of the esophagus. Pancreatic heterotopia can be complicated by ductal type pancreatic adenocarcinomas, by acinus cell carcinomas, by intraductal papillary mucinous neoplasias, and also by endocrine tumors. The present paper summarizes our current knowledge about heterotopias in atopographic clinico-pathological manner.

Combined Pancreatic and Gastric Heterotopia Presenting as a Gallbladder Mass: An Under-Recognized Cause of Post-Prandial Right Upper Quadrant Pain

Pancreatic and gastric heterotopias are defined as pancreatic and gastric tissue located outside their anatomic location without vascular or anatomical continuity with the pancreas proper or the stomach. These heterotopias have been described in the esophagus, stomach, and Meckel's diverticulum, however their presence in the gallbladder is rare. Heterotopias in the gallbladder can present with symptoms of acute and chronic cholecystitis, and perforation. 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 suspicion of acute cholecystitis. An 18-year-old woman presented with several years of nausea, non-biliary emesis and right upper quadrant pain. Her symptoms were most noticeable post prandial after a high fat meal. She was treated symptomatically for years with ondansetron, promethazine, NSAIDs, and sucralfate without relief. She denied any past medical or surgical history. A previous EGD was unremarkable and biopsies of the stomach and duodenum were negative for metaplasia and dysplasia. A right upper quadrant ultrasound showed a 9.6 mm polyp without sludge or stones. Cholecystectomy was performed and gross specimen showed a 0.9 x 0.7 x 0.6 cm rubbery pink polypoid nodule. Pathology revealed polyploid gastric and pancreatic heterotopia with focal intestinal metaplasia. There was no dysplasia and immunohistochemical stain for helicobacter pylori was negative. Mild chronic cholecystitis and cholesterolosis were present. The incidence of heterotopic pancreatic and gastric tissues ranges from 0.1 to 13 percent in the general population. Though the etiology is no yet well defined, theories suggest it results from an error during embryological development during rotation of the foregut. Acquired forms of heterotopia have been described, particularly gastric metaplasia. Though usually benign, malignant transformation can occur. Heterotopic pancreatic and gastric heterotopia in the gallbladder is difficult to diagnose preoperatively and is usually confirmed by histopathologic examination of the surgically resected specimen. Because pancreatic and gastric heterotopia is rare and often accompanied with nonspecific symptoms, delay in diagnosis occurs. In addition, awareness of this under reported condition by pathologist is important for its identification because should not be diagnosed as neoplastic disease, such as adenoma.Figure: Histology of the gallbladder mass showing combined pancreatic and gastric heterotopia with H&E stain at 20x magnification.Figure: Histology of the gallbladder mass showing combined pancreatic and gastric heterotopia with H&E stain at 400x magnification.Figure: Histology of the gallbladder mass showing combined pancreatic and gastric heterotopia with H&E stain at 400x magnification.

Efficacy of endoscopic ultrasound-guided fine-needle aspiration and core needle biopsy in the diagnosis of upper gastrointestinal submucosal lesions

Endoscopic ultrasonographically guided fine-needle aspiration (EUS-FNA) has been increasingly utilized to evaluate submucosal lesions of the upper gastrointestinal (UGI) tract. Our study aims to determine the efficacy of UGI EUS-FNA/core needle biopsy (CNB), including the frequency and cytomorphologic features of encountered submucosal lesions, and to investigate contributing factors including the role of rapid on-site evaluation (ROSE). We analyzed all UGI submucosal lesions diagnosed at our institution by EUS-FNA/CNB from September 2008 through August2015. During this 8-year study period, 94 patients underwent 110 UGI EUS-FNA/CNB, including 89 (81%) gastric, 11 (10%) duodenal, and 10 (9%) esophageal lesions. Twenty-seven (25%) were gastrointestinal stromal tumors (GISTs), followed by 13 (12%) leiomyomas, 5 (5%) schwannomas, 4 (4%) gastric adenocarcinomas, 3 (3%) neuroendocrine tumors (NETs), and 3 (3%) pancreatic heterotopias. All GISTs, leiomyomas, and NETs were ultimately diagnosed by EUS biopsies, as well as 75% of adenocarcinomas, 60% of schwannomas, and 33% of pancreatic heterotopias. The specificity of EUS-FNA/CNB for these 6 most commonly encountered lesions was 100%, with sensitivity of 82%. Sensitivity was 100% for esophageal and duodenal biopsies, and 80% and 75% for gastric and gastroesophageal procedures, respectively. Factors that contributed to poor yield included the lack of ROSE, small lesional size, lesion location and histology, and needle type. Neither number of needle passes nor operator experience appeared to influence specimen adequacy. EUS-FNA/CNB is an effective modality for diagnosing UGI submucosal lesions. Awareness of potential errors due to sampling of the bowel wall, lesional cystic degeneration, as well as pancreatic heterotopia and Brunner gland hamartoma is essential in order to avoid false diagnoses.

Clinical impact of duodenal pancreatic heterotopia \u2013 Is there a need for surgical treatment?

BackgroundPancreatic 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.MethodsA 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).ResultsA 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%.ConclusionsPH 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.

Pancreatic heterotopia presenting as acute cholecystitis in a 69-year-old male

Pancreatic heterotopia presenting as acute cholecystitis in a 69-year-old male

A Mass of Pancreatic and Gastric Heterotopia Causing a Small Bowel Obstruction in a 61-Year-Old Male

Heterotopic tissue is a congenital anomaly that has been previously reported. Gastric and pancreatic heterotopia are among the most studied ones. Herein, we describe a case of a combined pancreatic and gastric heterotopia that formed a mass and caused a small intestine obstruction in a 61-year-old male. We also did a brief literature review of cases with gastric and pancreatic heterotopia in adult patients.

Gastric Outlet Obstruction Caused by a Triple Lesion: Eosinophilic Gastroenteritis, Brunner’s Gland Hyperplasia and Pancreatic Heterotopia in Unison

Gastric outlet obstruction is a common entity described with numerous benign and malignant causes. Historically, benign causes include Peptic Ulcer disease (PUD); however recent cases have reported other causes including Eosinophilic Gastroenteritis (EGE), Brunner’s Gland Hyperplasia (BGH) and Pancreatic Heterotopia (PH). We present an unusual case of a 31 year old male patient with severe gastric outlet obstruction requiring surgical intervention caused by all three pathologies in unison – EGE, BGH and PH. The patient underwent surgical resection with distal gastrectomy and Billroth II reconstruction. Histology confirmed the three pathologies and post-operative recovery of the patient was uneventful.