Zollinger-Ellison syndrome(ZES)is characterized by gastric acid hypersecretion causing severe recurrent acid-related peptic disease.Excessive secretion of gastrin can now be effectively controlled with powerful proton...Zollinger-Ellison syndrome(ZES)is characterized by gastric acid hypersecretion causing severe recurrent acid-related peptic disease.Excessive secretion of gastrin can now be effectively controlled with powerful proton pump inhibitors,but surgical management to control gastrinoma itself remains controversial.Based on a thorough literature review,we design a surgical algorithm for ZES and list some significant consensus findings and recommendations:(1)For sporadic ZES,surgery should be routinely undertaken as early as possible not only for patients with a precisely localized diagnosis but also for those with negative imaging findings.The surgical approach for sporadic ZES depends on the lesion location(including the duodenum,pancreas,lymph nodes,hepatobiliary tract,stomach,and some extremely rare sites such as the ovaries,heart,omentum,and jejunum).Intraoperative liver exploration and lymphadenectomy should be routinely performed;(2)For multiple endocrine neoplasia type 1-related ZES(MEN1/ZES),surgery should not be performed routinely except for lesions>2 cm.An attempt to perform radical resection(pancreaticoduodenectomy followed by lymphadenectomy)can be made.The ameliorating effect of parathyroid surgery should be considered,and parathyroidectomy should be performed first before any abdominal surgery for ZES;and(3)For hepatic metastatic disease,hepatic resection should be routinely performed.Currently,liver transplantation is still considered an investigational therapeutic approach for ZES.Well-designed prospective studies are desperately needed to further verify and modify the current considerations.展开更多
AIM: To analyze the correlation of treatment method with the outcome of all the hepatic metastatic mela-noma (HMM) patients from our hospital.METHODS: There were altogether nine cases of HMM that had been treated in ... AIM: To analyze the correlation of treatment method with the outcome of all the hepatic metastatic mela-noma (HMM) patients from our hospital.METHODS: There were altogether nine cases of HMM that had been treated in the PUMCH hospital during the past 25 years, from December 1984 to February 2010. All of the cases developed hepatic metastasis from primary cutaneous melanoma. A retrospective review was performed on all the cases in order to draw informative conclusion on diagnosis and treatment in correlation with the prognosis. Clinical features including symptoms, signs, blood test results, Bultrasound and computed tomography (CT) imaging characteristics, and pathological data were analyzed in each case individually. A simple comparison was made on case by case basis instead of performing statistical analysis since the case numbers are low and patients were much diversified in each item that has been analyzed. Literatures on this subject were reviewed in order to draw a safe conclusion and found to be supportive to our finding in a much broad scope. RESULTS: There are six males and three females whose ages ranged 39-74 years old with an average of 58.8. Patients were either with or without symptoms at the time of diagnosis. The liver function and tumor marker exam were normal in all but one patient. The incidence of HMM does not affect liver function and was not related to virus infection status in the liver. Most of these HMM patients were also accompanied by the metastases of other locations, including lung, abdominal cavity, and cervical lymph nodes. Ultra-sound examinations showed lesions ranging 2-12 cm in diameter, with noor low-echo peripheral areola. Doppler showed blood flow appeared inside some tumors as well as in the surrounding area. CT image demonstrated low density without uniformed lesions, characterized with calcification in periphery, and en-hanced in the arterial phase. Contrast phase showed heterogeneous enhancement, with a density higher than normal liver tissue, which was especially appar-e展开更多
The management of hepatic metastatic disease from solid tumors in adults has been extensively described and resection of metastatic liver lesions from colorectal adenocarcinoma, renal adenocarcinoma, breast cancer, te...The management of hepatic metastatic disease from solid tumors in adults has been extensively described and resection of metastatic liver lesions from colorectal adenocarcinoma, renal adenocarcinoma, breast cancer, testicular cancer, and neuroendocrine tumors(NET) havedemonstrated therapeutic benefits in select patients. However, there are few reports in the literature on the management of hepatic metastatic disease in the pediatric and adolescent populations and the effectiveness of hepatic metastasectomy. This may be due to the much lower incidence of pediatric malignancies and the higher chemosensitivity of childhood tumors which make hepatic metastasectomy less likely to be required. We review liver involvement with metastatic disease from the main pediatric solid tumors, including neuroblastoma and Wilms tumor focusing on the management and treatment options. We also review other solid malignant tumors which may have liver metastases including germ cell tumors, gastrointestinal stromal tumors, osteosarcoma, desmoplastic small round cell tumors and NET. However, these histological subtypes are so rare in the pediatric and adolescent populations that the exact incidence and best management of hepatic metastatic disease are unknown and can only be extrapolated from adult series.展开更多
基金Supported by the National Natural Science Foundation,No.81773215the General Financial Grant from the China Postdoctoral Science Foundation,No.2017M610813
文摘Zollinger-Ellison syndrome(ZES)is characterized by gastric acid hypersecretion causing severe recurrent acid-related peptic disease.Excessive secretion of gastrin can now be effectively controlled with powerful proton pump inhibitors,but surgical management to control gastrinoma itself remains controversial.Based on a thorough literature review,we design a surgical algorithm for ZES and list some significant consensus findings and recommendations:(1)For sporadic ZES,surgery should be routinely undertaken as early as possible not only for patients with a precisely localized diagnosis but also for those with negative imaging findings.The surgical approach for sporadic ZES depends on the lesion location(including the duodenum,pancreas,lymph nodes,hepatobiliary tract,stomach,and some extremely rare sites such as the ovaries,heart,omentum,and jejunum).Intraoperative liver exploration and lymphadenectomy should be routinely performed;(2)For multiple endocrine neoplasia type 1-related ZES(MEN1/ZES),surgery should not be performed routinely except for lesions>2 cm.An attempt to perform radical resection(pancreaticoduodenectomy followed by lymphadenectomy)can be made.The ameliorating effect of parathyroid surgery should be considered,and parathyroidectomy should be performed first before any abdominal surgery for ZES;and(3)For hepatic metastatic disease,hepatic resection should be routinely performed.Currently,liver transplantation is still considered an investigational therapeutic approach for ZES.Well-designed prospective studies are desperately needed to further verify and modify the current considerations.
基金Supported by The National Natural Science Foundation of China, No. 30901453
文摘 AIM: To analyze the correlation of treatment method with the outcome of all the hepatic metastatic mela-noma (HMM) patients from our hospital.METHODS: There were altogether nine cases of HMM that had been treated in the PUMCH hospital during the past 25 years, from December 1984 to February 2010. All of the cases developed hepatic metastasis from primary cutaneous melanoma. A retrospective review was performed on all the cases in order to draw informative conclusion on diagnosis and treatment in correlation with the prognosis. Clinical features including symptoms, signs, blood test results, Bultrasound and computed tomography (CT) imaging characteristics, and pathological data were analyzed in each case individually. A simple comparison was made on case by case basis instead of performing statistical analysis since the case numbers are low and patients were much diversified in each item that has been analyzed. Literatures on this subject were reviewed in order to draw a safe conclusion and found to be supportive to our finding in a much broad scope. RESULTS: There are six males and three females whose ages ranged 39-74 years old with an average of 58.8. Patients were either with or without symptoms at the time of diagnosis. The liver function and tumor marker exam were normal in all but one patient. The incidence of HMM does not affect liver function and was not related to virus infection status in the liver. Most of these HMM patients were also accompanied by the metastases of other locations, including lung, abdominal cavity, and cervical lymph nodes. Ultra-sound examinations showed lesions ranging 2-12 cm in diameter, with noor low-echo peripheral areola. Doppler showed blood flow appeared inside some tumors as well as in the surrounding area. CT image demonstrated low density without uniformed lesions, characterized with calcification in periphery, and en-hanced in the arterial phase. Contrast phase showed heterogeneous enhancement, with a density higher than normal liver tissue, which was especially appar-e
文摘The management of hepatic metastatic disease from solid tumors in adults has been extensively described and resection of metastatic liver lesions from colorectal adenocarcinoma, renal adenocarcinoma, breast cancer, testicular cancer, and neuroendocrine tumors(NET) havedemonstrated therapeutic benefits in select patients. However, there are few reports in the literature on the management of hepatic metastatic disease in the pediatric and adolescent populations and the effectiveness of hepatic metastasectomy. This may be due to the much lower incidence of pediatric malignancies and the higher chemosensitivity of childhood tumors which make hepatic metastasectomy less likely to be required. We review liver involvement with metastatic disease from the main pediatric solid tumors, including neuroblastoma and Wilms tumor focusing on the management and treatment options. We also review other solid malignant tumors which may have liver metastases including germ cell tumors, gastrointestinal stromal tumors, osteosarcoma, desmoplastic small round cell tumors and NET. However, these histological subtypes are so rare in the pediatric and adolescent populations that the exact incidence and best management of hepatic metastatic disease are unknown and can only be extrapolated from adult series.