Case Of Heterotopic Pancreas Research Articles

A case of heterotopic pancreas mimicking an intestinal neuroendocrine tumor in 18F-DOPA PET-CT and a review of the literature

18-Fluoro-L-dihydroxyphenylalanine positron emission tomography/computed tomography (18F-DOPA PET-CT) is a nuclear medicine imaging modality indicated for the diagnosis and staging of neuroendocrine tumors (NETs), particularly for the midgut tumors, due to its excellent sensitivity and specificity. Its performance for the detection of foregut-derived NETs (duodenopancreas and proximal jejunum) and for the detection of hindgut-derived NETs is poor and inferior to PET somatostatin receptor imaging such as DOTATOC PET-CT and even inferior to somatostatin analog scintigraphy (octreoscan). There are few studies in the literature on heterotopic pancreas (HP) which is a rare entity, and which can be in some cases a false positive in 18F-DOPA PET-CT. We report a case of HP showing focal uptake on 18F-DOPA PET-CT mimicking an intestinal NET. This case suggests that HP should be included as a possible false positive on 18F-DOPA PET-CT.

Pancreatic heterotopia: An overview of a lesion often mimicking neuroendocrine tumour

Pancreatic tissue that has no direct or vascular connection to the main body of the pancreas constitutes ectopic pancreas or heterotopic pancreas (HP). Majority are located in the upper gastrointestinal tract (GIT). HP may be mistaken for neoplasms like neuroendocrine tumour (NET) and adenocarcinoma on endoscopy as well as on histopathology. We report six cases of HP along with review of literature; three of them were misdiagnosed as NET. To the best of our knowledge, this is the first case series of HP from India, rest being case reports. Histopathological features of six cases of HP were analyzed. Variables examined were clinical presentation, age at diagnosis, location, histopathologic type and immunohistochemical profile. Two cases each were seen in duodenum and Meckel’s diverticulum while one case each was seen in stomach and an atretic segment of ileum, a site never reported previously. There were five males and one female with age ranging from one day to 48 years. Patients with gastric and duodenal HP were symptomatic. On histopathology, four cases were intramuscular and two were submucosal. Four cases were of Type I and two cases were of type II. HP should be considered in the differential diagnosis of submucosal upper GI lesions. Awareness, high degree of suspicion and thorough histopathological examination are necessary to arrive at a correct diagnosis. Immunohistochemistry for chromogranin and synaptophysin is useful for confirming the islets especially on small endoscopic biopsies. HP must be considered in the differential diagnosis of submucosal upper GI lesions. Knowledge about this entity, high degree of suspicion and thorough histopathological examination help in arriving at a correct diagnosis and excluding mimics.

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.

A Case of Heterotopic Pancreas in the Gallbladder Diagnosed as a Result of Acute Cholecystitis

A Case of Heterotopic Pancreas in the Gallbladder Diagnosed as a Result of Acute Cholecystitis

Heterotopic pancreas causing jejunal obstruction in a neonate

Abstract Heterotopic pancreas (HP) is a rare entity. HP is rarely diagnosed during the neonatal period, and only a few neonatal cases of HP causing gastrointestinal obstruction have been reported. We herein describe a rare case of HP causing jejunal obstruction in the early neonatal period and discuss the association of an inflammatory process in this case.

Heterotopic pancreas in the omphalomesenteric duct remnant in a 9-month-old girl: a case report and literature review

BackgroundHeterotopic pancreas most commonly occurs in the upper gastrointestinal tract of adults, usually as an incidental finding. It seldom occurs at the umbilicus, and even rarely in the pediatric age group.Case presentationHere we present a case of heterotopic pancreatic tissue in the omphalomesenteric duct remnant of a 9-month-old baby girl. She presented with redness at the base of the umbilicus associated with occasional mild wetness. A urachal fistula was suspected by ultrasound. Histology from subsequent resection revealed fibrous tissue with heterotopic pancreatic tissue and accompanying small bowel mucosa. The patient’s umbilical redness resolved after the surgery.ConclusionsUpon literature search, we found only 17 other cases of heterotopic pancreas reported in the umbilicus. They described a high male to female ratio, frequent association with omphalomesenteric duct remnant and presentation of umbilical discharge. The Heinrich system is frequently used to classify heterotopic pancreas into 3 types, based on the presence of acini, islets and ducts. Several mechanisms have been proposed on the pathogenesis of heterotopic pancreas, including misplacement, metaplasia and totipotent cell theories. Heterotopic pancreas can manifest clinically with diseases of the pancreas, including malignant transformation, reported as high as 12.7% in a series. Awareness of this finding in the biopsy aids the suitable treatment decisions for the patient.

Heterotopic Pancreatic Tissue in Gall Bladder: A Rare Entity

Introduction: Pancreatic heterotopia is a rare pathological entity and its localization in gall bladder is extremely rare. It is mostly asymptomatic incidental finding and rarely gives rise to complications. It can be associated with incidental finding or can be symptomatic or may cause complication. Heterotopic pancreas (HP) also referred to as ectopic pancreas, pancreatic choristoma, or pancreatic rest, is defined as the aberrantly located pancreatic tissue in an anomalous location without any anatomic, vascular, or neural continuity with the main body of the normal pancreas. The most common location for HP is the duodenum, upper jejunum and stomach. Case report: A 35 year male was admitted to the Surgical Department of Era’s Lucknow Medical College and Hospital for an elective laparoscopic cholecystectomy. He had complains of sporadic flatulence after meals and belching. His gall bladder was resected post an USG revealing Gall stones and sent for histopathology where he was diagnosed as a case of heterotopic pancreas in the GB neck with chronic cholecystitis. Discussion: Localization of pancreatic heterotopia in gall bladder is the second most prevalent pancreatic anomaly but an extremely rare finding. The incidence in gastrointestinal tract is estimated to be from 0.55% to 13.7% on autopsy, and 0.2% in laparatomy. Most commonly these are incidental findings but some studies have implicated them as causative factors in the genesis of cholecystitis, obstructions etc. Conclusion: HP of the gallbladder may cause clinical symptoms such as cholecystitis as well as complications which can be fatal and hence this condition should be taken into consideration in patients with symptomatic gallbladder disease.

Interventional endoscopic ultrasound for a symptomatic pseudocyst secondary to gastric heterotopic pancreas.

Heterotopic pancreas (HP) is a relatively uncommon entity that is defined as pancreatic tissue without a true anatomical or vascular connection to the pancreas. HP does not cause symptoms in most cases but can occasionally produce various manifestations, including nausea, vomiting, abdominal pain, and even heterotopic pancreatitis. Here, we report an unusual case in which heterotopic pancreatitis complicated by the formation of a pseudocyst that caused gastric outlet obstruction was diagnosed based on serum hyperamylasemia and findings from endoscopic ultrasonography (EUS)-guided fine needle aspiration (EUS-FNA) cytology. EUS-guided single pigtail stent insertion was successfully performed for recurrent gastric outlet obstruction. The patient has remained healthy and symptom-free during 4 years of surveillance. In the context of the relevant literature, the described case is a rare case of HP complicated by a pseudocyst treated via EUS-FNA and stent insertion.

An Unusual Location for Pancreatic Heterotopia: The Gastroesophageal Junction

: Heterotopic pancreas (HP) is defined as pancreatic tissue found outside the pancreas. When symptoms appear, they are often due to inflammation, obstruction, and malignant transformation. The most common location is the stomach, especially the antrum. HP in the esophagus is uncommon, with only a handful of cases reported in pediatric patients. It rarely occurs in the adult population.We present an atypical case of HP at the gastroesophageal junction (GE) in a middleaged female. Case: A 59 year old African American female was referred for iron deficiency anemia. Patient had recurrent cough. She denied symptoms of dyspepsia. Physical exam was unremarkable. The white blood cell was 9.7 (3.8-10.6), blood Hg 11.9 g/dL, HCT 34.4% with an MCV of 77.8 L, and platelet count was 246. Stool occult testing negative. An esophagogastroduodenoscopy revealed a small, broad-based polyp about 3-4 mm in diameter without umbilication, or evidence of bleeding (Figure 1). It was glimpsed at the GE junction on introduction of the endoscope, and confirmed on a retroflexed view (Figure 2). A biopsy of the polyp was obtained with cold forceps, and sent for histopathologic examination which revealed focal pancreatic acinar cell metaplasia/heterotopia completely surrounded by gastric cardia-type mucosa with chronic active gastritis, acute cryptitis (Figure 3). There was no evidence of intestinal metaplasia or dysplasia, or esophageal squamous mucosa. Because the polyp was located at the GE junction, the origin could have been esophageal or gastric. However, because the surrounding tissue histologically was entirely gastric, a diagnosis of HP of the stomach at the GE junction was assigned. The patient had been asymptomatic and did not present with complications, a follow up plan for endoscopic surveillance was proposed.Figure 1Figure 2Figure 3Discussion: The incidence of HP cases has been reported to be 2 to 15%, with the majority found incidentally during surgery or post-mortem autopsies. Endoscopically, HPs grossly appear as firm white to yellow lobulated nodules and range in size almost 0.2 to 4 cm. Our case was unusual in that it did not have the characteristic umbilication, nor discoloration, but rather a nodular polypoid appearance, and was located not in the atrum or body, but at the GE junction. In a symptomatic patient, resection is performed after other causes are ruled out. In an asymptomatic patient, with benign gross and histological features, lesions are managed conservatively.

Mutational spectrum of intraepithelial neoplasia in pancreatic heterotopia

Heterotopic pancreatic parenchyma recapitulates the normal pancreas in extrapancreatic locations and, on rare occasions, can even give rise to pancreatic adenocarcinoma. The genetic signatures of pancreatic adenocarcinoma and its precursor lesions are well characterized. We explored the genetic alterations in precursor lesions (intraductal papillary mucinous neoplasms [IPMN], pancreatic intraepithelial neoplasia [PanIN]) in patients with pancreatic heterotopias but without concomitant pancreatic ductal adenocarcinomas. This allowed us to determine whether the stereotypical dysplasia--infiltrating carcinoma sequence also occurs in these extrapancreatic foci. Seven cases of heterotopic pancreas with ductal precursor lesions were identified. These included 2 IPMNs with focal high-grade dysplasia and 5 PanINs with low- to moderate-grade dysplasia (PanIN grades 1-2). Neoplastic epithelium was microdissected and genomic DNA was extracted. Sequencing of commonly mutated hotspots (KRAS, TP53, CDKN2A, SMAD4, BRAF, and GNAS) in pancreatic ductal adenocarcinoma and its precursor lesions was performed. Both IPMNs were found to have KRAS codon 12 mutations. The identification of KRAS mutations suggests a genetic pathway shared with IPMN of the pancreas. No mutations were identified in our heterotopic PanINs. One of the possible mechanisms for the development of dysplasia in these lesions is field effect. At the time of these resections, there was no clinical or pathologic evidence of a prior or concomitant pancreatic lesion. However, a clinically undetectable lesion is theoretically possible. Therefore, although a field effect cannot be excluded, there was no evidence for it in this study.

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.

腹痛と嚢胞性病変の増大縮小を繰り返した上行結腸間膜内異所性膵の1例

症例は19歳，女性．右下腹部痛を主訴に当院紹介となった．腹部CTで上行結腸内側に径34mmの嚢胞性病変を認め，上行結腸憩室炎の診断で保存的加療を行い軽快した．しかし同様の症状による入退院を繰り返したため，虫垂病変に伴う膿瘍形成を疑い，腹腔鏡下虫垂切除術を施行したが，その後も2カ月毎に症状の増悪と軽快を繰り返した．経過中，症状増悪時には嚢胞性病変の増大と炎症所見を認め，軽快時には病変は縮小していた．繰り返す腹痛の原因を上行結腸間膜内の嚢胞性病変と断定し，初診から2年6カ月後，根治治療目的に腹腔鏡下回盲部切除を施行した．病理組織学的検査で，嚢胞性病変は拡張した導管であり，その周囲に膵腺房細胞を認め，Heinrich II型異所性膵と診断した．上行結腸間膜内に発生した異所性膵の本邦での報告は本例を除き1例のみであり，繰り返す腹痛の原因となった上行結腸間膜内異所性膵について，文献的考察を加え報告する．

A Case of Heterotopic Pancreas in the Stomach

A Case of Heterotopic Pancreas in the Stomach

Heterotopic pancreas complicated by pseudocyst in the gastric wall diagnosed by endoscopic ultrasound-guided fine needle aspiration

Heterotopic pancreas is usually asymptomatic and does not change throughout the patient's life, but sometimes it can present symp­toms and complications, which are rarely discrete in the literature. We present here a case of heterotopic pancreas in the gastric wall complicated with pseudocyst, and suggest that heterotopic pancreatic pseudocyst should be included in the differential diagnosis of gastric wall cysts.

Sa1640 Thirty Cases of Heterotopic Pancreas: Experience From a Single High-Volume Medical Center in China

Sa1640 Thirty Cases of Heterotopic Pancreas: Experience From a Single High-Volume Medical Center in China

Triple intussusception involving heterotopic pancreatic tissue: a case report

IntroductionIntussusception involving heterotopic pancreatic tissue is a rare condition where a portion of the bowel telescopes into an adjacent segment with intraluminal pancreatic tissue as the lead point. Cases of heterotopic pancreas are most often described in the upper intestinal tract, particularly the stomach.Case presentationWe present the case of a five-month-old boy of Caucasian ethnicity suffering acute abdominal pain and vomiting with an abdominal mass in the upper right quadrant. Work-up including ultrasound scan confirmed the intussusception. Repeated attempts at radiological reduction and two laparoscopic procedures were performed within 24 hours, which eventually led to the diagnosis of a triple intussusception.ConclusionTo our knowledge, such a case of triple intussusception involving isolated heterotopic pancreatic tissue is previously unreported.

6604 Exploring the role of CA 19–9 level as a predictor of survival in patients with advanced pancreatic adenocarcinoma

Heterotopic pancreatic parenchyma recapitulates the normal pancreas in extrapancreatic locations and, on rare occasions, can even give rise to pancreatic adenocarcinoma. The genetic signatures of pancreatic adenocarcinoma and its precursor lesions are well characterized. We explored the genetic alterations in precursor lesions (intraductal papillary mucinous neoplasms [IPMN], pancreatic intraepithelial neoplasia [PanIN]) in patients with pancreatic heterotopias but without concomitant pancreatic ductal adenocarcinomas. This allowed us to determine whether the stereotypical dysplasia--infiltrating carcinoma sequence also occurs in these extrapancreatic foci. Seven cases of heterotopic pancreas with ductal precursor lesions were identified. These included 2 IPMNs with focal high-grade dysplasia and 5 PanINs with low- to moderate-grade dysplasia (PanIN grades 1-2). Neoplastic epithelium was microdissected and genomic DNA was extracted. Sequencing of commonly mutated hotspots (KRAS, TP53, CDKN2A, SMAD4, BRAF, and GNAS) in pancreatic ductal adenocarcinoma and its precursor lesions was performed. Both IPMNs were found to have KRAS codon 12 mutations. The identification of KRAS mutations suggests a genetic pathway shared with IPMN of the pancreas. No mutations were identified in our heterotopic PanINs. One of the possible mechanisms for the development of dysplasia in these lesions is field effect. At the time of these resections, there was no clinical or pathologic evidence of a prior or concomitant pancreatic lesion. However, a clinically undetectable lesion is theoretically possible. Therefore, although a field effect cannot be excluded, there was no evidence for it in this study.

Heterotopic pancreas in children: review of the literature and report of 12 cases

Heterotopic pancreas (HP) is rarely recognized during surgery. Many reports concerning this anomaly are simple case reports. We herein review our experiences with HP. We retrospectively investigated cases of HP from April 1975 to September 2006. We discussed the frequencies in the laparotomized patients, and patient's age, gender, operative indication, location of HP, post-operative diagnosis and pathology. A total of 12 patients with HP, 3 boys and 9 girls, aged 1 day to 10 years of age were investigated. Ten patients had one pattern of HP and the remaining two had more than one. The locations of the HP were as follows: Meckel's diverticulum, 4; stomach, 3; duodenum, 3; jejunum, 3; and ileum, 2. Only in one patient HP had caused an intussusception. The remaining 11 cases were identified incidentally during the operation. In 11 of 12 patients, HP was removed without post-operative complications. Classification of pathologies according to Heinrich is as follows: type I, 4; type II, 4; type III, 2 and unknown, 2. Patients with HP are usually asymptomatic. However, HP caused an intussusception in our series. Incidental HPs should be removed whenever identified during laparotomy.

An unusual cause of cholecystitis: Heterotopic pancreatic tissue in the gallbladder

Gallbladder localization of heterotopic pancreas (HP) is uncommon and very rarely gives rise to symptoms. Herein we report a case of HP found in the gallbladder neck presented with signs and symptoms of cholecystitis. The patient was a 40-year old male, suffering from epigastric pain, abdominal fullness and fever. On physical examination, the right upper abdomen was tender with a positive Murphy's sign. Ultrasonographic examination showed a hydropic gallbladder without stones and he underwent a cholecystectomy. Pathological examination revealed an intramural nodule (9 mm) in the neck region which is consisted of acini, ducts and islet cells of an aberrant pancreatic tissue. Although HP is encountered rarely in the gallbladder and is found incidentally during pathological studies, this case emphasizes that HP might cause symptoms and present clinically as cholecystitis. For this reason, in patients presenting with symptomatic gallbladder diseases, including cholecystitis without any other pathology, HP should be taken into consideration before it is diagnosed as "idiopathic".

上行結腸癌の手術中に偶然発見された空腸迷入すいの１例

上行結腸癌手術中に偶然発見された空腸迷入膵の１例を経験したので報告する．症例は73歳女性．便秘，腹部膨満感，左下腹部痛のため精査目的で当科入院となった．腹部X線検査で下行結腸より口側に多量のガス貯留を伴う著明な腸管拡張を認めた．大腸内視鏡検査では下行結腸に約10cmにわたる虚血性変化と思われる狭窄部位を認めた．また，上行結腸起始部に２型の腫瘤が認められ病理組織学的検査でadenocarcinomaと診断され回盲部切除が施行された．開腹時の腹腔内検索でTreitz靱帯より130cm肛側空腸の腸間膜対側に直径約１cmの腫瘤を触知したため空腸部分切除を追加した．術後病理組織学的検査で小腸腫瘤はHeinrich分類III型の迷入膵と診断された．開腹手術時の腹腔内臓器検索の際には本症例の存在も考慮に入れるべきであると考えられた．