Background:There are no clearly defined indications for pancreas-preserving duodenectomy.The present study aimed to analyze postoperative morbidity and the outcomes of patients undergoing pancreaspreserving duodenecto...Background:There are no clearly defined indications for pancreas-preserving duodenectomy.The present study aimed to analyze postoperative morbidity and the outcomes of patients undergoing pancreaspreserving duodenectomy.Methods:Patients undergoing pancreas-preserving duodenectomy from April 2008 to May 2020 were included.We divided the series according to indication:scenario 1,primary duodenal tumors;scenario 2,tumors of another origin with duodenal involvement;and scenario 3,emergency duodenectomy.Results:We included 35 patients.Total duodenectomy was performed in 1 patient of adenomatous duodenal polyposis,limited duodenectomy in 7,and third+fourth duodenal portion resection in 27.The indications for scenario 1 were gastrointestinal stromal tumor(n=13),adenocarcinoma(n=4),neuroendocrine tumor(n=3),duodenal adenoma(n=1),and adenomatous duodenal polyposis(n=1);scenario 2:retroperitoneal desmoid tumor(n=2),recurrence of liposarcoma(n=2),retroperitoneal paraganglioma(n=1),neuroendocrine tumor in pancreatic uncinate process(n=1),and duodenal infiltration due to metastatic adenopathies of a germinal tumor with digestive hemorrhage(n=1);and scenario 3:aortoenteric fistula(n=3),duodenal trauma(n=1),erosive duodenitis(n=1),and biliopancreatic limb ischemia(n=1).Severe complications(Clavien-Dindo≥IIIb)developed in 14%(5/35),and postoperative mortality was 3%(1/35).Conclusions:Pancreas-preserving duodenectomy is useful in the management of primary duodenal tumors,and is a technical option for some tumors with duodenal infiltration or in emergency interventions.展开更多
BACKGROUND: Duodenal gastrointestinal stromal tumors, which are rare, comprise 3%-5% of all gastrointestinal stromai tumors. We present a case of a metastatic duodenal gastrointestinal stromal tumor that was successfu...BACKGROUND: Duodenal gastrointestinal stromal tumors, which are rare, comprise 3%-5% of all gastrointestinal stromai tumors. We present a case of a metastatic duodenal gastrointestinal stromal tumor that was successfully treated by simultaneous tight hemihepatectomy and pancreaticoduodenectomy. METHODS: A 50-year-old woman was admitted to our department for the treatment of a possible metastatic duodenal gastrointestinal stromal tumor (GIST). At laparotomy a large duodenal tumor was found displacing the head of the pancreas. A 3 cm in diameter lesion in the posterior aspect of segment Ⅷ of the liver was also noted. Simultaneous right hepatectomy and pancreaticoduodenectomy were performed. RESULTS: Histological examination revealed a high grade metastatic duodenal GIST strongly positive for c-kit, CD34, and vimentin. The patient had no additional therapy. A follow-up of 21 months showed that the patient is very well and there is no evidence of recurrent diseases. CONCLUSIONS: Malignant stromai tumors of the duodenum are rarely encountered. They are usually slow growing, and may be amenable to curative surgery, even after occurrence of metastases. Resection of localized liver metastasis is still advocated when feasible, since imatinib does not provide a complete or long-term response. Combined surgical resection is an efficacious treatment for patients with metastatic duodenal gastrointestinal stromal tumor.展开更多
BACKGROUND Gastrointestinal(GI)lipomas are benign submucosal tumors of mature adipocytes that arise mainly in the colon and stomach,sometimes in the ileum and jejunum,and rarely in the duodenum.Patients with symptomat...BACKGROUND Gastrointestinal(GI)lipomas are benign submucosal tumors of mature adipocytes that arise mainly in the colon and stomach,sometimes in the ileum and jejunum,and rarely in the duodenum.Patients with symptomatic lipomas require endoscopic or surgical treatment.Spontaneous expulsion of lipomas after biopsy is a rare condition that has limited case reports.CASE SUMMARY A 56-year-old man presented to our hospital with intermittent postprandial epigastric fullness.Esophagogastroduodenoscopy(EGD)revealed a 10-mm soft yellowish submucosal lesion with the“pillow sign,”located in the second portion of duodenum.Endoscopic ultrasonography(EUS)using a 12-MHz catheter probe showed a hyperechoic,homogenous,and round solid lesion(OLYMPUS EUS EUME2,UM-DP12-25R,12-MHz radial miniprobe,Olympus Corporation,Tokyo,Japan).Deep biopsy was performed using the bite-on-bite technique with forceps.Histological examination was compatible with submucosal lipoma.The lesion spontaneously expelled 12 d after the biopsy.Follow-up EUS performed after 2 mo confirmed this condition.CONCLUSION Deep biopsy could lead to spontaneous GI lipoma expulsion.This might be the first step in lipoma diagnosis and treatment.展开更多
AIM To investigate predictors of perforation after endoscopic resection(ER) for duodenal neoplasms without a papillary portion.METHODS This was a single-center, retrospective, cohort study conducted between April 2003...AIM To investigate predictors of perforation after endoscopic resection(ER) for duodenal neoplasms without a papillary portion.METHODS This was a single-center, retrospective, cohort study conducted between April 2003 and September 2014. A total of 54 patients(59 lesions) underwent endoscopic mucosal resection(EMR)(n = 36) and endoscopic submucosal dissection(ESD)(n = 23). Clinical features, outcomes, and predictors of perforation were investigated.RESULTS Cases of perforation occurred in eight(13%) patients(95%CI: 4.7%-22.6%). Three ESD cases required sur-gical management because they could not be repaired by clipping. Delayed perforation occurred in two ESD cases, which required surgical management, although both patients underwent prophylactic clipping. All patients with perforation who required surgery had no postoperative complications and were discharged at anaverage of 13.2 d after ER. Perforation after ER showed a significant association with a tumor size greater than20 mm(P = 0.014) and ESD(P = 0.047).CONCLUSION ESD for duodenal neoplasms exceeding 20 mm may be associated with perforation. ESD alone is not recom-mended for tumor treatment, and LECS should be con-sidered as an alternative.展开更多
The presentation,subtype,and macroscopic images of lymphoma vary depending on the site of the tumor within the gastrointestinal tract.We searched PubMed for publications between January 1,2012 and October 10,2022,and ...The presentation,subtype,and macroscopic images of lymphoma vary depending on the site of the tumor within the gastrointestinal tract.We searched PubMed for publications between January 1,2012 and October 10,2022,and retrieved 130 articles relating to duodenal lymphoma.A further 22 articles were added based on the manual screening of relevant articles,yielding 152 articles for full-text review.The most predominant primary duodenal lymphoma was follicular lymphoma.In this review,we provide an update of the diagnosis and management of representative lymphoma subtypes occurring in the duodenum:Follicular lymphoma,diffuse large B-cell lymphoma,extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue,mantle cell lymphoma,and Tcell lymphomas.展开更多
AIM: To evaluate the results of segmental duodenectomy (SD) and pancreaticoduodenectomy (PD) for duodenal gastrointestinal stromal tumor (GIST) and help clinicians with surgical management. METHODS: All patients who u...AIM: To evaluate the results of segmental duodenectomy (SD) and pancreaticoduodenectomy (PD) for duodenal gastrointestinal stromal tumor (GIST) and help clinicians with surgical management. METHODS: All patients who underwent surgery for non-metastatic GIST of the duodenum in a single institution since 2000 were prospectively followed up. Seven patients (median age 51 years, range: 41-73 years) were enrolled: five underwent SD and two underwent PD. RESULTS: All the patients had a complete resection (R0), with no postoperative morbidity and mortality. Among the SD group, GIST was classified as low risk in two patients, intermediate risk in two, and high risk in one, according to the Fletcher scale, (vs two high risk patients in the PD group). With a median followup of 41 (18-85) mo, disease-free survival (DFS) rateswere 100% after SD and 0% after PD (P < 0.05). The median DFS was 13 mo in the PD group. CONCLUSION: Whenever associated with clear surgical margins, SD is a reliable and curative option for most duodenal GISTs, and is compatible with longterm DFS.展开更多
BACKGROUND: Gastrointestinal stromal tumors are the most common mesenchymal tumors of the gastrointestinal tract and occur rarely in the duodenum. Splenic angiosarcoma is an aggressive neoplasm with an extremely poor ...BACKGROUND: Gastrointestinal stromal tumors are the most common mesenchymal tumors of the gastrointestinal tract and occur rarely in the duodenum. Splenic angiosarcoma is an aggressive neoplasm with an extremely poor prognosis. METHODS: We report a case of a 70-year-old man hospitalized for abdominal pain in the upper quadrants, dyspepsia and nausea, previously treated for Hodgkin lymphoma 30 years ago. Abdominal CT showed a solid nodular lesion in the third portion of the duodenum, the presence of retropancreatic, aortic and caval lymph nodes, and four nodular splenic masses. 111 In-octreotide scintigraphy revealed pathological tissue accumulation in the duodenal region, and in the retropancreatic, retroduodenal, aortic and caval lymph nodes, suggesting a nonfunctioning neuroendocrine peripancreatic tumor. RESULTS: At exploratory laparotomy, an exophytic soft tumor was found originating from the third portion of the duodenum. Pancreas-preserving duodenectomy with duodenojejunostomy, splenectomy and lymphnodectomy of retropancreatic aortic and caval lymph nodes were performed. Pathological evaluation and immunohistochemical studies showed the presence of a duodenal gastrointestinal stromal tumor with low mitotic activity and a well-differentiated angiosarcoma localized to the spleen and invading lymph nodes.CONCLUSIONS: We speculated that the angiosarcoma and duodenal gastrointestinal stromal tumors of this patient were due to the treatment of Hodgkin lymphoma with radiotherapy 30 years ago. Pancreas-preserving segmental duodenectomy can be used to treat non-malignant neoplasms of the duodenum and avoid extensive surgery. Splenectomy is the treatment of choice for localized angiosarcomas but a strict follow-up is mandatory because of the possibility of recurrence.展开更多
BACKGROUND: The trauma caused by pancreatoduode- nectomy for periampullary carcinoma of vater is often se- vere and extensive. The purpose of this study was to eva- luate the effect of extended local resection in the ...BACKGROUND: The trauma caused by pancreatoduode- nectomy for periampullary carcinoma of vater is often se- vere and extensive. The purpose of this study was to eva- luate the effect of extended local resection in the treatment of periampullary carcinoma of vater. METHODS: The extra-hepaticobiliary tract, the confluence of the pancreatic and biliary duct, vater ampulla and duo- denal papilla were resected en bloc in 8 patients with peri- ampullary carcinoma from 1995 to 1998. RESULTS: One patient died perioperatively. Duodenal ob- struction developed postoperatively in one of 7 survived pa- tients and was relieved after reoperation. All the 7 patients were followed up for more than 6 months without recur- rence. CONCLUSION: Extended local resection fulfils the task of radical treatment of periampullary malignancy.展开更多
In order to study the relationship of p53 gene mutation with the occurrence and prognosis of cancer of small intestine, expression of p53 protein was immunohistochemically examined. The results showed that p53 protein...In order to study the relationship of p53 gene mutation with the occurrence and prognosis of cancer of small intestine, expression of p53 protein was immunohistochemically examined. The results showed that p53 protein expression was high in 75% of small intestine cancer, and positive in 21.1% of the tissues close to cancer. In 7 cases of small intestinal adenoma, only one was immunoreactive. Sixteen samples of normal tissue of the intestine didn't show expression of p53protein. The study also found that the degree of p53protein expression was significantly correlated with that of tumor cell differentiation, invasion, metastasis and prognosis.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
基金supported by grants from the Institut de Investigació Biomèdica de Bellvitge(IDIBELL Foundation)the CERCA Programme/Generalitat de Catalunya。
文摘Background:There are no clearly defined indications for pancreas-preserving duodenectomy.The present study aimed to analyze postoperative morbidity and the outcomes of patients undergoing pancreaspreserving duodenectomy.Methods:Patients undergoing pancreas-preserving duodenectomy from April 2008 to May 2020 were included.We divided the series according to indication:scenario 1,primary duodenal tumors;scenario 2,tumors of another origin with duodenal involvement;and scenario 3,emergency duodenectomy.Results:We included 35 patients.Total duodenectomy was performed in 1 patient of adenomatous duodenal polyposis,limited duodenectomy in 7,and third+fourth duodenal portion resection in 27.The indications for scenario 1 were gastrointestinal stromal tumor(n=13),adenocarcinoma(n=4),neuroendocrine tumor(n=3),duodenal adenoma(n=1),and adenomatous duodenal polyposis(n=1);scenario 2:retroperitoneal desmoid tumor(n=2),recurrence of liposarcoma(n=2),retroperitoneal paraganglioma(n=1),neuroendocrine tumor in pancreatic uncinate process(n=1),and duodenal infiltration due to metastatic adenopathies of a germinal tumor with digestive hemorrhage(n=1);and scenario 3:aortoenteric fistula(n=3),duodenal trauma(n=1),erosive duodenitis(n=1),and biliopancreatic limb ischemia(n=1).Severe complications(Clavien-Dindo≥IIIb)developed in 14%(5/35),and postoperative mortality was 3%(1/35).Conclusions:Pancreas-preserving duodenectomy is useful in the management of primary duodenal tumors,and is a technical option for some tumors with duodenal infiltration or in emergency interventions.
文摘BACKGROUND: Duodenal gastrointestinal stromal tumors, which are rare, comprise 3%-5% of all gastrointestinal stromai tumors. We present a case of a metastatic duodenal gastrointestinal stromal tumor that was successfully treated by simultaneous tight hemihepatectomy and pancreaticoduodenectomy. METHODS: A 50-year-old woman was admitted to our department for the treatment of a possible metastatic duodenal gastrointestinal stromal tumor (GIST). At laparotomy a large duodenal tumor was found displacing the head of the pancreas. A 3 cm in diameter lesion in the posterior aspect of segment Ⅷ of the liver was also noted. Simultaneous right hepatectomy and pancreaticoduodenectomy were performed. RESULTS: Histological examination revealed a high grade metastatic duodenal GIST strongly positive for c-kit, CD34, and vimentin. The patient had no additional therapy. A follow-up of 21 months showed that the patient is very well and there is no evidence of recurrent diseases. CONCLUSIONS: Malignant stromai tumors of the duodenum are rarely encountered. They are usually slow growing, and may be amenable to curative surgery, even after occurrence of metastases. Resection of localized liver metastasis is still advocated when feasible, since imatinib does not provide a complete or long-term response. Combined surgical resection is an efficacious treatment for patients with metastatic duodenal gastrointestinal stromal tumor.
基金Supported by the Medical Technology and Education of Zhejiang Province of China,No. Y202146136
文摘BACKGROUND Gastrointestinal(GI)lipomas are benign submucosal tumors of mature adipocytes that arise mainly in the colon and stomach,sometimes in the ileum and jejunum,and rarely in the duodenum.Patients with symptomatic lipomas require endoscopic or surgical treatment.Spontaneous expulsion of lipomas after biopsy is a rare condition that has limited case reports.CASE SUMMARY A 56-year-old man presented to our hospital with intermittent postprandial epigastric fullness.Esophagogastroduodenoscopy(EGD)revealed a 10-mm soft yellowish submucosal lesion with the“pillow sign,”located in the second portion of duodenum.Endoscopic ultrasonography(EUS)using a 12-MHz catheter probe showed a hyperechoic,homogenous,and round solid lesion(OLYMPUS EUS EUME2,UM-DP12-25R,12-MHz radial miniprobe,Olympus Corporation,Tokyo,Japan).Deep biopsy was performed using the bite-on-bite technique with forceps.Histological examination was compatible with submucosal lipoma.The lesion spontaneously expelled 12 d after the biopsy.Follow-up EUS performed after 2 mo confirmed this condition.CONCLUSION Deep biopsy could lead to spontaneous GI lipoma expulsion.This might be the first step in lipoma diagnosis and treatment.
文摘AIM To investigate predictors of perforation after endoscopic resection(ER) for duodenal neoplasms without a papillary portion.METHODS This was a single-center, retrospective, cohort study conducted between April 2003 and September 2014. A total of 54 patients(59 lesions) underwent endoscopic mucosal resection(EMR)(n = 36) and endoscopic submucosal dissection(ESD)(n = 23). Clinical features, outcomes, and predictors of perforation were investigated.RESULTS Cases of perforation occurred in eight(13%) patients(95%CI: 4.7%-22.6%). Three ESD cases required sur-gical management because they could not be repaired by clipping. Delayed perforation occurred in two ESD cases, which required surgical management, although both patients underwent prophylactic clipping. All patients with perforation who required surgery had no postoperative complications and were discharged at anaverage of 13.2 d after ER. Perforation after ER showed a significant association with a tumor size greater than20 mm(P = 0.014) and ESD(P = 0.047).CONCLUSION ESD for duodenal neoplasms exceeding 20 mm may be associated with perforation. ESD alone is not recom-mended for tumor treatment, and LECS should be con-sidered as an alternative.
文摘The presentation,subtype,and macroscopic images of lymphoma vary depending on the site of the tumor within the gastrointestinal tract.We searched PubMed for publications between January 1,2012 and October 10,2022,and retrieved 130 articles relating to duodenal lymphoma.A further 22 articles were added based on the manual screening of relevant articles,yielding 152 articles for full-text review.The most predominant primary duodenal lymphoma was follicular lymphoma.In this review,we provide an update of the diagnosis and management of representative lymphoma subtypes occurring in the duodenum:Follicular lymphoma,diffuse large B-cell lymphoma,extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue,mantle cell lymphoma,and Tcell lymphomas.
文摘AIM: To evaluate the results of segmental duodenectomy (SD) and pancreaticoduodenectomy (PD) for duodenal gastrointestinal stromal tumor (GIST) and help clinicians with surgical management. METHODS: All patients who underwent surgery for non-metastatic GIST of the duodenum in a single institution since 2000 were prospectively followed up. Seven patients (median age 51 years, range: 41-73 years) were enrolled: five underwent SD and two underwent PD. RESULTS: All the patients had a complete resection (R0), with no postoperative morbidity and mortality. Among the SD group, GIST was classified as low risk in two patients, intermediate risk in two, and high risk in one, according to the Fletcher scale, (vs two high risk patients in the PD group). With a median followup of 41 (18-85) mo, disease-free survival (DFS) rateswere 100% after SD and 0% after PD (P < 0.05). The median DFS was 13 mo in the PD group. CONCLUSION: Whenever associated with clear surgical margins, SD is a reliable and curative option for most duodenal GISTs, and is compatible with longterm DFS.
文摘BACKGROUND: Gastrointestinal stromal tumors are the most common mesenchymal tumors of the gastrointestinal tract and occur rarely in the duodenum. Splenic angiosarcoma is an aggressive neoplasm with an extremely poor prognosis. METHODS: We report a case of a 70-year-old man hospitalized for abdominal pain in the upper quadrants, dyspepsia and nausea, previously treated for Hodgkin lymphoma 30 years ago. Abdominal CT showed a solid nodular lesion in the third portion of the duodenum, the presence of retropancreatic, aortic and caval lymph nodes, and four nodular splenic masses. 111 In-octreotide scintigraphy revealed pathological tissue accumulation in the duodenal region, and in the retropancreatic, retroduodenal, aortic and caval lymph nodes, suggesting a nonfunctioning neuroendocrine peripancreatic tumor. RESULTS: At exploratory laparotomy, an exophytic soft tumor was found originating from the third portion of the duodenum. Pancreas-preserving duodenectomy with duodenojejunostomy, splenectomy and lymphnodectomy of retropancreatic aortic and caval lymph nodes were performed. Pathological evaluation and immunohistochemical studies showed the presence of a duodenal gastrointestinal stromal tumor with low mitotic activity and a well-differentiated angiosarcoma localized to the spleen and invading lymph nodes.CONCLUSIONS: We speculated that the angiosarcoma and duodenal gastrointestinal stromal tumors of this patient were due to the treatment of Hodgkin lymphoma with radiotherapy 30 years ago. Pancreas-preserving segmental duodenectomy can be used to treat non-malignant neoplasms of the duodenum and avoid extensive surgery. Splenectomy is the treatment of choice for localized angiosarcomas but a strict follow-up is mandatory because of the possibility of recurrence.
文摘BACKGROUND: The trauma caused by pancreatoduode- nectomy for periampullary carcinoma of vater is often se- vere and extensive. The purpose of this study was to eva- luate the effect of extended local resection in the treatment of periampullary carcinoma of vater. METHODS: The extra-hepaticobiliary tract, the confluence of the pancreatic and biliary duct, vater ampulla and duo- denal papilla were resected en bloc in 8 patients with peri- ampullary carcinoma from 1995 to 1998. RESULTS: One patient died perioperatively. Duodenal ob- struction developed postoperatively in one of 7 survived pa- tients and was relieved after reoperation. All the 7 patients were followed up for more than 6 months without recur- rence. CONCLUSION: Extended local resection fulfils the task of radical treatment of periampullary malignancy.
文摘In order to study the relationship of p53 gene mutation with the occurrence and prognosis of cancer of small intestine, expression of p53 protein was immunohistochemically examined. The results showed that p53 protein expression was high in 75% of small intestine cancer, and positive in 21.1% of the tissues close to cancer. In 7 cases of small intestinal adenoma, only one was immunoreactive. Sixteen samples of normal tissue of the intestine didn't show expression of p53protein. The study also found that the degree of p53protein expression was significantly correlated with that of tumor cell differentiation, invasion, metastasis and prognosis.
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.