BACKGROUND Zollinger–Ellison syndrome(ZES)results from hypersecretion of gastrin from pancreatic or duodenal neuroendocrine tumors,commonly referred to as gastrinomas.The high levels of gastrin lead to a typical pres...BACKGROUND Zollinger–Ellison syndrome(ZES)results from hypersecretion of gastrin from pancreatic or duodenal neuroendocrine tumors,commonly referred to as gastrinomas.The high levels of gastrin lead to a typical presentation involving watery diarrhea and multiple ulcers in the duodenum.Here,we have presented the rare case of a patient with ZES and absence of hypergastrinemia as well as an atypical location of gastrinoma.CASE SUMMARY A 72-year-old woman presented with the typical clinical manifestations of ZES,including upper abdominal pain,significant watery diarrhea,and acidic liquid vomitus.Surprisingly,however,she did not have an increased level of serum gastrin.In addition,there was no evidence of gastrinoma or any other ulcerogenic tumor.Esophagogastroduodenoscopy was conducted to examine the upper digestive tract.Revised diagnoses were considered,and an individualized treatment plan was developed.The patient responded to antacid medication while experiencing intermittent,recurring bouts of ZES.18F-AlF-NOTAoctreotide positron emission tomography(18F-OC PET)/computed tomography(CT)helped locate the tumor.Postoperative pathology and immunohistochemistry results suggested that the tumor was a gastrinoma located at an unconventional site.CONCLUSION This present case study demonstrates the possibility of ZES-like manifestation in patients with absence of hypergastrinemia.18F-OC PET/CT is a relatively new imaging technique that can be applied for diagnosing even tiny gastrinomas that are atypical in terms of location.展开更多
Zollinger-Ellison syndrome(ZES)associated with pancreatic or duodenal gastrinoma is characterized by gastric acid hypersecretion,which typically leads to gastroesophageal reflux disease,recurrent peptic ulcers,and chr...Zollinger-Ellison syndrome(ZES)associated with pancreatic or duodenal gastrinoma is characterized by gastric acid hypersecretion,which typically leads to gastroesophageal reflux disease,recurrent peptic ulcers,and chronic diarrhea.As symptoms of ZES are nonspecific and overlap with other gastrointestinal disorders,the diagnosis is often delayed with an average time between the onset of symptoms and final diagnosis longer than 5 years.The critical step for the diagnosis of ZES is represented by the initial clinical suspicion.Hypergastrinemia is the hallmark of ZES;however,hypergastrinemia might recognize several causes,which should be ruled out in order to make a final diagnosis.Gastrin levels>1000 pg/mL and a gastric pH below 2 are considered to be diagnostic for gastrinoma;some specific tests,including esophageal pH-recording and secretin test,might be useful in selected cases,although they are not widely available.Endoscopic ultrasound is very useful for the diagnosis and the local staging of the primary tumor in patients with ZES,particularly in the setting of multiple endocrine neoplasia type 1.Some controversies about the management of these tumors also exist.For the localized stage,the combination of proton pump inhibitory therapy,which usually resolves symptoms,and surgery,whenever feasible,with curative intent represents the hallmark of gastrinoma treatment.The high expression of somatostatin receptors in gastrinomas makes them highly responsive to somatostatin analogs,supporting their use as anti-proliferative agents in patients not amenable to surgical cure.Other medical options for advanced disease are super-imposable to other neuroendocrine neoplasms,and studies specifically focused on gastrinomas only are scant and often limited to case reports or small retrospective series.The multidisciplinary approach remains the cornerstone for the proper management of this composite disease.Herein,we reviewed available literature about gastrinoma-associated ZES with a specific focus on differential diagnosis,providing potential diagnostic and therapeutic algorithms.展开更多
BACKGROUND Gastrinoma is characterized by an excessive release of gastrin,leading to hypersecretion of gastric acid,subsequently resulting in recurrent peptic ulcers,chronic diarrhea,and even esophageal strictures.Thi...BACKGROUND Gastrinoma is characterized by an excessive release of gastrin,leading to hypersecretion of gastric acid,subsequently resulting in recurrent peptic ulcers,chronic diarrhea,and even esophageal strictures.This case report aims to improve awareness and facilitate early diagnosis and treatment of gastrinoma by presenting a rare case of gastrinoma with refractory benign esophageal stricture(RBES).Additionally,it highlights the persistent challenges that gastroenterologists encounter in managing RBES.CASE SUMMARY This case demonstrates a patient with gastrinoma who developed RBES and complete esophageal obstruction despite management with maximal acid suppressive therapy,multiple endoscopic bougie dilations and endoscopic incisional therapy(EIT).CONCLUSION It is essential to diagnose gastrinoma as early as possible,as inadequately controlled acid secretion over an extended period increases the risk of developing severe esophageal strictures.In patients with esophageal strictures causing complete luminal obstruction,blind reopening EIT presents challenges and carries a high risk of perforation.展开更多
Primary hepatic gastrinoma is a rare disease, with fewer than 40 cases reported in the medical literature. Because it is located in an organ in which metastases are common, its diagnosis is difficult. We report a case...Primary hepatic gastrinoma is a rare disease, with fewer than 40 cases reported in the medical literature. Because it is located in an organ in which metastases are common, its diagnosis is difficult. We report a case of a 19 years old male patient with a history of gastric ulcers since the age of nine. Following gastric surgery, an antrectomy and a vagotomy, there was some alleviation of symptoms. Subsequently, the patient reported various intermittent episodes of diarrhea, diffuse abdominal pain, and vomiting. The patient underwent tomography, which revealed the presence of a hepatic mass measuring 19.5 cm × 12.5 cm × 17 cm. Primary hepatic gastrinoma was diagnosed based on laboratory examinations that indicated hypergastrinemia and a positron emission tomography/magnetic resonance study with somatostatin analogue that confirmed the liver as the primary site. After hepatic trisegmentectomy(Ⅱ, Ⅲ, Ⅳ, Ⅴ, Ⅷ), the patient's symptoms improved. The case is notable for the presence of a rare tumor with uncommon dimensions.展开更多
基金National Natural Science Foundation of China,No.82100612Zhejiang Medical Health Science and Technology Plan Project,No.2021KY1116。
文摘BACKGROUND Zollinger–Ellison syndrome(ZES)results from hypersecretion of gastrin from pancreatic or duodenal neuroendocrine tumors,commonly referred to as gastrinomas.The high levels of gastrin lead to a typical presentation involving watery diarrhea and multiple ulcers in the duodenum.Here,we have presented the rare case of a patient with ZES and absence of hypergastrinemia as well as an atypical location of gastrinoma.CASE SUMMARY A 72-year-old woman presented with the typical clinical manifestations of ZES,including upper abdominal pain,significant watery diarrhea,and acidic liquid vomitus.Surprisingly,however,she did not have an increased level of serum gastrin.In addition,there was no evidence of gastrinoma or any other ulcerogenic tumor.Esophagogastroduodenoscopy was conducted to examine the upper digestive tract.Revised diagnoses were considered,and an individualized treatment plan was developed.The patient responded to antacid medication while experiencing intermittent,recurring bouts of ZES.18F-AlF-NOTAoctreotide positron emission tomography(18F-OC PET)/computed tomography(CT)helped locate the tumor.Postoperative pathology and immunohistochemistry results suggested that the tumor was a gastrinoma located at an unconventional site.CONCLUSION This present case study demonstrates the possibility of ZES-like manifestation in patients with absence of hypergastrinemia.18F-OC PET/CT is a relatively new imaging technique that can be applied for diagnosing even tiny gastrinomas that are atypical in terms of location.
文摘Zollinger-Ellison syndrome(ZES)associated with pancreatic or duodenal gastrinoma is characterized by gastric acid hypersecretion,which typically leads to gastroesophageal reflux disease,recurrent peptic ulcers,and chronic diarrhea.As symptoms of ZES are nonspecific and overlap with other gastrointestinal disorders,the diagnosis is often delayed with an average time between the onset of symptoms and final diagnosis longer than 5 years.The critical step for the diagnosis of ZES is represented by the initial clinical suspicion.Hypergastrinemia is the hallmark of ZES;however,hypergastrinemia might recognize several causes,which should be ruled out in order to make a final diagnosis.Gastrin levels>1000 pg/mL and a gastric pH below 2 are considered to be diagnostic for gastrinoma;some specific tests,including esophageal pH-recording and secretin test,might be useful in selected cases,although they are not widely available.Endoscopic ultrasound is very useful for the diagnosis and the local staging of the primary tumor in patients with ZES,particularly in the setting of multiple endocrine neoplasia type 1.Some controversies about the management of these tumors also exist.For the localized stage,the combination of proton pump inhibitory therapy,which usually resolves symptoms,and surgery,whenever feasible,with curative intent represents the hallmark of gastrinoma treatment.The high expression of somatostatin receptors in gastrinomas makes them highly responsive to somatostatin analogs,supporting their use as anti-proliferative agents in patients not amenable to surgical cure.Other medical options for advanced disease are super-imposable to other neuroendocrine neoplasms,and studies specifically focused on gastrinomas only are scant and often limited to case reports or small retrospective series.The multidisciplinary approach remains the cornerstone for the proper management of this composite disease.Herein,we reviewed available literature about gastrinoma-associated ZES with a specific focus on differential diagnosis,providing potential diagnostic and therapeutic algorithms.
文摘BACKGROUND Gastrinoma is characterized by an excessive release of gastrin,leading to hypersecretion of gastric acid,subsequently resulting in recurrent peptic ulcers,chronic diarrhea,and even esophageal strictures.This case report aims to improve awareness and facilitate early diagnosis and treatment of gastrinoma by presenting a rare case of gastrinoma with refractory benign esophageal stricture(RBES).Additionally,it highlights the persistent challenges that gastroenterologists encounter in managing RBES.CASE SUMMARY This case demonstrates a patient with gastrinoma who developed RBES and complete esophageal obstruction despite management with maximal acid suppressive therapy,multiple endoscopic bougie dilations and endoscopic incisional therapy(EIT).CONCLUSION It is essential to diagnose gastrinoma as early as possible,as inadequately controlled acid secretion over an extended period increases the risk of developing severe esophageal strictures.In patients with esophageal strictures causing complete luminal obstruction,blind reopening EIT presents challenges and carries a high risk of perforation.
文摘Primary hepatic gastrinoma is a rare disease, with fewer than 40 cases reported in the medical literature. Because it is located in an organ in which metastases are common, its diagnosis is difficult. We report a case of a 19 years old male patient with a history of gastric ulcers since the age of nine. Following gastric surgery, an antrectomy and a vagotomy, there was some alleviation of symptoms. Subsequently, the patient reported various intermittent episodes of diarrhea, diffuse abdominal pain, and vomiting. The patient underwent tomography, which revealed the presence of a hepatic mass measuring 19.5 cm × 12.5 cm × 17 cm. Primary hepatic gastrinoma was diagnosed based on laboratory examinations that indicated hypergastrinemia and a positron emission tomography/magnetic resonance study with somatostatin analogue that confirmed the liver as the primary site. After hepatic trisegmentectomy(Ⅱ, Ⅲ, Ⅳ, Ⅴ, Ⅷ), the patient's symptoms improved. The case is notable for the presence of a rare tumor with uncommon dimensions.
