Ectopic Pancreatic Tissue Research Articles

7204 An Unexpected Cause of Ga-68 Dotatate PET/CT Somatostatin Receptor Avidity in a Pediatric Patient with Hereditary Paraganglioma-Pheochromocytoma Syndrome due to an SDHA Mutation

Abstract Disclosure: K. Leopold: None. S. Polites: None. L. Binkovitz: None. L. Casas: None. A.N. Lteif: None. Title: An Unexpected Cause of Ga-68 Dotatate PET/CT Somatostatin Receptor Avidity in a Pediatric Patient with Hereditary Paraganglioma-Pheochromocytoma Syndrome due to an SDHA Mutation. Introduction: Individuals with Succinate Dehydrogenase (SDHx) gene mutations have an increased risk of paragangliomas and pheochromocytomas beginning in childhood.Clinical Case: A 9 year old male with Cornelia de Lange syndrome and a paternally inherited SDHA pathogenic gene variant (c1534C>T) presented with worsening migraine headaches and skin pallor with activity. 24 hour urine fractionated metanephrines were mildly elevated, with metanephrine 122 mcg/24h (29-92 mcg/24 h), normetanephrine 187 mcg/24 h (34-169 mcg/24 h), and total metanephrine 309 mcg/24 h (47-223 mcg/24 h). Ga-68 DOTATATE PET/CT showed a focus of moderately increased radiotracer activity in the anterior aspect of the distal gastric antrum with a max SUV 8.2 corresponding to a 9x7 mm nodule on CT in the same area, suggestive of underlying somatostatin receptor positive lesion such as a paraganglioma. MRI with contrast completed to further characterize the lesion showed no visible mass corresponding to the focal area of radiotracer uptake on DOTATATE PET/CT. Repeat 24 h urine metanephrines continued to show mild elevation of metanephrine (138 mcg/24h), normetanephrine (200 mcg/24h), and total metanephrines (338 mcg/24h). 24 hour urine catecholamines, 5-Hydroxyindoleacetic acid, and homovanillic acid were normal. An upper endoscopic ultrasound was completed which confirmed the presence of a lesion near the stomach but not arising from the stomach wall and not consistent with a gastrointestinal stromal tumor. CT enterography with IV contrast showed a 6x6 mm enhancing mass within the distal right lateral gastric wall. Due to ongoing symptoms, mild elevations in urine fractionated metanephrines, and a clear mass on US and CT, the patient underwent laparoscopic abdominal exploration where a 14x14x13 mm mass with surrounding gastric and duodenal serosa was excised with primary closure of the bowel. Pathology showed ectopic pancreatic tissue in the wall of the small bowel and stomach. Repeat Ga-68 DOTATATE PET/CT 5 months following surgery showed resolution of the distal gastric somatostatin receptor avidity. Clinical Lesson: This is the first case of ectopic pancreatic tissue mimicking a paraganglioma in a pediatric patient with hereditary paraganglioma-pheochromocytoma syndrome. False positive imaging should be considered in patients with SDHx mutations when the uptake is atypical in location and when the elevation in urine metanephrines is mild. Presentation: 6/3/2024

Dual Heterotropic Tissue Associated with a Type IV A Choledochal Cyst: A Rare Case Report

Abstract A choledochal cyst (CC) is one of the rare congenital diseases of the biliary tract and is more common in females. The association of ectopic pancreatic and splenic tissue with CC is an extremely rare condition. An 11-year-old girl child presented with a vague abdominal lump and intermittent dull abdominal pain for 5 months with early satiety and weight loss in the current case scenario. Her laboratory findings were normal. A type IV CC was discovered on ultrasonography, and the distal common bile duct displayed significant cystic dilatation on magnetic resonant cholangiopancreatography. After the CC was surgically removed, it was sent for histopathological examination. Sections revealed dual heterotropic elements, which included pancreatic and splenic tissues in a CC. It was very uncommon for both to exist independently in the CC and in this instance, both were observed together. This will be the first documented case, in which the CC contained dual ectopic tissue from the spleen and pancreas. Ectopic splenic tissue in the CC has never been documented in the literature. It is crucial to be aware of this entity when splenic or pancreatic tissue experiences changes due to inflammation, even though its clinical significance is uncertain. In the ectopic rests, bleeding, blockage, and malignant transformation are the possible outcomes.

Laparoscopic management of small bowel obstruction secondary to a mesodiverticular band of a Meckel's diverticulum in an adult: A case report and literature review.

The mesodiverticular band (MDB) of a Meckel's diverticulum (MD) is a rare, yet notable etiology of small bowel obstruction (SBO) in adults. Due to the nonspecific symptoms and challenging diagnosis thereof, preoperative clinical suspicion and strategic management are crucial for achieving optimal outcomes. Therefore, we presented a case in which laparoscopic surgery was strategically performed to alleviate ileus, due to a preoperative diagnosis of SBO, suspected to be secondary to an MD with a concomitant MDB. A 32-year-old male patient presented with a half-day's duration of epigastric pain, abdominal distension, and tenderness, resulting in the working diagnosis of SBO. Initial non-contrast computed tomography (CT) revealed SBO without signs of strangulation, postulated to be caused by an MD and concomitant MDB, resulting in conservative management. The symptoms persisted, necessitating contrast-enhanced CT. However, the dilated bowel loop suggestive of an MD that had been observed on non-contrast CT could not be confirmed on contrast-enhanced CT. Decompression therapy using a long tube provided minimal relief, prompting laparoscopic surgery on the 5th day post-admission for diagnostic and therapeutic purposes. An MD resection effectively relieved the SBO. The histopathological analysis revealed a true diverticulum with ectopic pancreatic tissue, confirming the diagnosis of an MD. At the band site, vascular and neural structures were encased in a sheath, consistent with the remnants of the vitelline duct mesentery; and histopathologically diagnosed as an MDB. The postoperative course was uneventful, and the patient was discharged on the 9th day, postoperatively. Decompression therapy and strategic laparoscopic surgery based on the preoperative working diagnosis of SBO yielded favorable outcomes, highlighting the importance of the early clinical suspicion of an MD and a concomitant MDB, as the etiology of SBO. The imaging variability and rarity of an MD in adults emphasizes the need for a heightened awareness and an accurate diagnosis for optimal management. Early intervention should be deliberated for patients with suspected intestinal ischemia. However, this case accentuates the clinical implications of strategic planning and employing minimally invasive techniques in the management of an MD-related SBO in adults.

A Rare Case of Ectopic Pancreatic Tissue in Type 1 Choledochal Cyst with Cholelithiasis and Choledocholithiasis in a 4-Year-Old Female - Case Report

A Rare Case of Ectopic Pancreatic Tissue in Type 1 Choledochal Cyst with Cholelithiasis and Choledocholithiasis in a 4-Year-Old Female - Case Report

Differentiation of ectopic pancreatic tissue from gastric tumor

Differentiation of ectopic pancreatic tissue from gastric tumor

New frontiers in ectopic pancreatic tissue management.

The pancreatic development variations are relatively frequent but are often overlooked in clinical practice. This is due to the fact that they do not present with a distinct clinical picture and are usually asymptomatic. It also refers to the ectopic pancreatic tissue in the stomach. This anomaly can be diagnosed in any part of the digestive system, but it is mostly seen in the upper gastrointestinal tract, especially in the stomach, duodenum and jejunum. The management of this condition has evolved due to the development of minimally invasive procedures.

Gastric Outlet Obstruction Caused by Gastric Ectopic Pancreas With Pseudocyst Formation

Ectopic pancreas refers to presence of pancreatic tissue in locations other than the pancreas (the usual anatomical site). This condition is often asymptomatic but can occasionally cause abdominal pain or other gastrointestinal symptoms. Ectopic pancreatic tissue is mainly observed in the stomach and duodenum and may be accompanied by complications, such as hemorrhage, pancreatitis, gastric outlet obstruction due to pseudocyst, and malignant transformation. We report a rare case of gastric ectopic pancreas accompanied by a pseudocyst that led to gastric outlet obstruction, which improved after endoscopic ultrasonography guided fine-needle aspiration in a young woman with habitual excessive alcohol consumption.

Ectopic pancreatic adenocarcinoma in Meckel’s diverticulum: a case report

BackgroundMalignant neoplasms arising from Meckel’s diverticulum are rare and an adenocarcinoma in Meckel’s diverticulum originating from ectopic pancreatic tissue is even rarer. Herein, we report a patient with an ectopic pancreatic adenocarcinoma in Meckel’s diverticulum who was successfully treated with surgery and chemotherapy.Case presentationA woman in her sixties presented to another hospital with abdominal pain. Plain computed tomography suggested an abdominal tumor and she was referred to our hospital. Enhanced computed tomography revealed a 23-mm low-density tumor in the abdominal cavity. Surgery was performed with a tentative diagnosis of a mesenteric tumor, such as a gastrointestinal stromal tumor, schwannoma, or lymphoma. First, we inspected the peritoneal cavity with a laparoscope. This revealed numerous nodules in the small bowel mesentery, suggesting peritoneal dissemination. A 20-mm-diameter white tumor was found in the small intestine and diagnosed as a small intestinal cancer. The small intestine was partially resected laparoscopically through a small skin incision. The patient’s postoperative course was uneventful, and she was discharged on postoperative day 9. Pathological examination revealed well-differentiated adenocarcinoma in the small intestine. The tumor had developed from a sac-like portion protruding toward the serosal side and had a glandular structure lined with flattened atypical cells. Neither pancreatic acinar cells nor islets of Langerhans were evident, suggesting a Heinrich type 3 ectopic pancreas. The final diagnosis was an adenocarcinoma originating from an ectopic pancreas in Meckel’s diverticulum. After a smooth recovery, the patient commenced chemotherapy for pancreatic cancer.ConclusionsWe present a very rare case of ectopic pancreatic carcinoma in Meckel’s diverticulum.

Ileo-Ileal Intussusception Caused by a Meckel’s Diverticulum With Ectopic Pancreatic Tissue: A Case Report

Ileo-Ileal Intussusception Caused by a Meckel’s Diverticulum With Ectopic Pancreatic Tissue: A Case Report

Unusual presentation of ectopic pancreas

Background : Ectopic pancreas defined as a pancreatic tissue lacking vascular or anatomical continuity with the Main body of pancreas. The majority of cases is a symptomaic, however they can present with bleeding or luminal obstruction. Herein, we present a case that had ectopic pancreatic tissue in the mesentery of proximal jejunum mimicking gastrointestinal stromal tumor. Case presentation : We present a case of a 24-year-old Saudi female, married who presented to the emergency room because of lower central abdominal pain radiating to the flanks for 9 days with on and off fever, one day diarrhea and urinary symptoms. She was conscious, coherent, afebrile and ambulatory with vital signs in the ER revealed an oral temperature of 36.5oC, heart rate of 81 bpm, respiratory rate of 18 per minute, BP of 121/75 mmHg, and SpO2 of 99%. Abdominal examination revealed soft and lax tenderness in the lower central abdomen and left flanks. Conclusion : Ectopic pancreatitis should be taken into account in a patient who has chronic abdominal pain with a normal native pancreas according to imaging results. Small intestinal tumors, whether detected on imaging investigations or accidentally found after surgery conducted for another reason, should be diagnosed with ectopic pancreas in mind.

Pathologic examination of ectopic pancreatic tissue in the gallbladder: A case report

Abstract Introduction/Objective Ectopic pancreatic tissue (EPT) is an entity defined by the presence of pancreatic tissue at its non-physiological site, while co-existing with original tissue in its correct anatomical location. While EPT is a rare process in locations such as the stomach, duodenum, and esophagus; it is even more uncommon for it to occur in the gallbladder. To our knowledge, fewer than thirty cases of EPT in the gallbladder have been reported. The following case report presents a 47-year-old woman with ectopic pancreatic tissue in the gallbladder. Methods/Case Report A 47-year-old female presented to our emergency department with her third episode of colicky right upper quadrant pain. Ultrasound imaging demonstrated cholelithiasis with additional ill-defined findings of the gallbladder wall, which included but were not limited to acute emphysematous cholecystitis and chronic cholecystitis with features of porcelain gallbladder. Laparoscopic cholecystectomy was performed without complications. Pathologic evaluation of the specimen demonstrated a cystic structure at the fundus of the gallbladder with coexisting EPT, fundic-type adenomyomatosis, and cholelithiasis. Results (if a Case Study enter NA) NA Conclusion Ectopic pancreatic tissue is a rare incidental finding which is often confused for a malignant process. While it is more commonly seen in the gastrointestinal tract from the esophagus to the duodenum, it can present in other sites such as in this case. Currently, there is no specific imaging method to determine the presence of EPT. Ultrasonography is nonspecific and EPT can be confused for a number of conditions ranging from benign to malignant. Gross examination is fairly unremarkable, however microscopic examination is a straightforward diagnosis. While it is a benign process, malignant transformation can occur and pathologists should be aware of this possibility. For this reason, additional sections may be recommended to rule out occult malignancy.

A Rare Case of Gastric Ectopic Pancreatic Tissue - An Incidental finding

Ectopic pancreas is an infrequent lesion presenting as a nodule or a submucosal mass in the gastrointestinal tract. Depending upon its anatomical location, it can cause clinical symptoms like abdominal pain, intussusceptions, or obstruction, or it can remain clinically silent. Though radiologic diagnostic tools are available, the preoperative diagnosis is tough and is seldom made. We report a case of a 61-year-old male with the chief complaint of pain in the abdomen for 15 days. Advanced diagnostic tools like Contrast enhanced CT scan can characterise the submucosal mass in the stomach and able to confirm the nature of the lesion, whether it was non-neoplastic, benign, or malignant. Therefore, surgical excision was done. The final confirmatory diagnosis of ectopic pancreas in the stomach was made on histopathologic examination.

S4029 Ectopic Pancreatic Tissue Leading to Intussusception of the Small Bowel

S4029 Ectopic Pancreatic Tissue Leading to Intussusception of the Small Bowel

Exome sequencing findings in children with annular pancreas.

Annular pancreas (AP) is a congenital defect of unknown cause in which the pancreas encircles the duodenum. Theories include abnormal migration and rotation of the ventral bud, persistence of ectopic pancreatic tissue, and inappropriate fusion of the ventral and dorsal buds before rotation. The few reported familial cases suggest a genetic contribution. We conducted exome sequencing in 115 affected infants from the California birth defects registry. Seven cases had a single heterozygous missense variant in IQGAP1, five of them with CADD scores >20; seven other infants had a single heterozygous missense variant in NRCAM, five of them with CADD scores >20. We also looked at genes previously associated with AP and found two rare heterozygous missense variants, one each in PDX1 and FOXF1. IQGAP1 and NRCAM are crucial in cell polarization and migration. Mutations result in decreased motility which could possibly cause the ventral bud to not migrate normally. To our knowledge, this is the first study reporting a possible association for IQGAP1 and NRCAM with AP. Our findings of rare genetic variants involved in cell migration in 15% of our population raise the possibility that AP may be related to abnormal cell migration.

A Rare Case of Duodenal Obstruction: Annular Pancreas in a 22 Years Old Patient: Case Report

Annular pancreas is a rare congenital condition in which a ring of ectopic pancreatic tissue partially or completely surrounds the second part of the duodenum [1]. This Congenital anomaly is usually presented in infants and newborn, rarely in adult life. It can present in a wide range of clinical severities making it difficult to diagnose. CT scan can illustrate the pancreatic tissue encircling the duodenum. Surgery still remains necessary to confirm diagnosis and bypassing the obstructed segment. We report the case of a 22 years old female patient who presented with history of vomiting and weight loss causing a stature weight growth delay, the diagnosis was made by the CT scan. Then the patient was scheduled for a gastroenteroanastomosis. The evolution was favorable.

A Rare Adenocarcinoma of Ectopic Pancreatic from Duodenum: A Case Report

Ectopic pancreas is a rare disease in which pancreatic tissue appears outside the pancreas. Malignant transformation of ectopic pancreatic tissue is uncommon. We present a case of ectopic pancreatic malignant transformation from the first part of the duodenum. A 68 year old male was referred to our hospital with recurrent upper abdominal pain for 3 months.

Pancreatic head cancer with gastrointestinal bleeding as the initial manifestation: a case report and literature review

Gastrointestinal bleeding (GIB) as the initial symptom is rare in patients with pancreatic cancer, which is prone to misdiagnosis and should be paid attention to by clinicians. Here, we present a patient with pancreatic head cancer whose first symptom was only severe upper gastrointestinal bleeding, which initially led to misdiagnosis. However, subsequent imaging revealed a mass in the pancreatic head which was considered a malignant tumor. After surgical resection, the pathology confirmed that the mass in the pancreatic head was pancreatic ductal adenocarcinoma, and the duodenal bulb’s full thickness was infiltrated. A literature review found that pancreatic cancer can present gastrointestinal bleeding by invading the digestive tract and blood vessels, left-sided portal hypertension (LSPH), digestive tract metastasis, and canceration of ectopic pancreatic tissue in the digestive tract. For these cases, clinicians should utilize multidisciplinary diagnosis and treatment based on digestive endoscopy, imaging, interventional therapy, and surgery to control bleeding, identify etiology, and remove tumors.

Acinar cell carcinoma of gastric ectopic pancreas origin: a case report and literature review

BackgroundPrimary pancreatic-type acinar cell carcinoma of the stomach is extremely rare, often misdiagnosed, and of unclear origin.Case presentationWe report the case of a primary pure pancreatic-type acinar cell carcinoma of the stomach in a 58-year-old woman. This is the first reported case to exhibit residual ectopic pancreatic tissue adjacent to the tumor serving as evidence for the origin of the carcinoma. Furthermore, we summarized the clinicopathological features of pancreatic-type acinar cell carcinoma of the stomach in order to further understand this solid tumor.ConclusionsPrimary pancreatic-type acinar cell carcinoma of the stomach is rare. Data on tumors of this histological type are still relatively scarce, and more in-depth research is needed to elucidate their molecular biological characteristics and prognosis.

Endoscopic enucleation of gastric choristoma in a child

Introduction. In pediatric practice, one may encounter various volumetric formations of the stomach, both benign and malignant. The verification and localization of the neoplasma is the most difficult issue in everyday work. Most often, benign tumors are represented by a rounded submucosal formation, covered with an intact mucous membrane. The most common formations that can be found in a child are leiomyomas. Objective is to present the first experience of endoscopic submucosal dissection and enucleation of the choristoma in a child. Clinical observation. The girl was admitted for inpatient treatment with complaints of prolonged (2 years) abdominal syndrome and mass formation of the antrum of the stomach. According to CT data, a voluminous soft tissue formation 8 × 7 × 12 mm in size was revealed in the antrum of the stomach, which does not accumulate a contrast agent. When performing esophagogastroduodenoscopy and endosonography, a submucosal formation of the prepyloric stomach was visualized, with dimensions of 7 × 10 mm. The macroscopic picture of the tumor corresponded to gastric leiomyoma. However, given the clinical picture, it was impossible to exclude the chorous stomach. Thus, the diagnosis was formulated: volumetric formation of the stomach. Leiomyoma? Choristoma? In the course of further treatment, surgical treatment was performed, in the amount of submucosal enucleation of the formation. According to the morphological study, the remote formation corresponded to the ectopic pancreatic tissue. Conclusion. Choristoma does not always have a “classic” appearance with retraction in the center, and the clinical picture is of great importance in the verification of neoplasma. Endoscopic submucosal removal of gastric choristoma is a minimally invasive and effective method of surgical treatment that can be used in children.

Intussusception causing bowel obstruction in a child due to ectopic pancreas: A rare case report and literature review

Introduction: Ectopic pancreas is a congenital anomaly in which the pancreatic tissue is located abnormally, lacking anatomical continuity with the main pancreas. This is not an uncommon anomaly, however the preoperative diagnosis is difficult and the majority of cases are diagnosed histologically after surgery. Ectopic pancreas is commonly found in the upper gastrointestinal tract (including stomach, duodenum and jejunum); rarer in the ileum. The main symptoms of ectopic pancreas in children include gastrointestinal bleeding, vomiting, recurrent abdominal pain, and intussusception. In this article, we report a case of a 4-year-old girl who was diagnosed with ectopic pancreas causing intussusception complicated by bowel obstruction. The patient was treated by laparotomy and recovered uneventfully. Case report: 4-year-old girl with a history of recurrent intussusception admitted with abdominal pain and vomiting. Abdominal ultrasound and computed tomography showed an intussusception complicated by bowel obstruction. The child underwent laparotomy with segmental intestinal resection and was diagnosed with intussusception due to ectopic pancreatic tissue in the ileal wall based on histopathological findings. After surgery, the child recovered uneventfully. Conclusion: Ectopic pancreas is common in the gastrointestinal tract, difficult to diagnose by imaging modalities. Most of cases are diagnosed histologically after sugery. Imaging methods, particularly abdominal ultrasound and computed tomography, play an important role in diagnosing the complications of ectopic pancreas such as intussusception and bowel obstruction. Key words: ectopic pancreas, recurrent intussusception, bowel obstruction