Insulinomas are the most frequent type of functional pancreatic neuroendocrine tumors with a variety of neuroglycopenic and autonomic symptoms and welldefined diagnostic criteria;however,prediction of their clinical b...Insulinomas are the most frequent type of functional pancreatic neuroendocrine tumors with a variety of neuroglycopenic and autonomic symptoms and welldefined diagnostic criteria;however,prediction of their clinical behavior and early differentiation between benign and malignant lesions remain a challenge.The comparative studies between benign and malignant cases are limited,suggesting that short clinical history,early hypoglycemia during fasting,high proinsulin,insulin,and C-peptide concentrations raise suspicion of malignancy.Indeed,malignant tumors are larger with higher mitotic count and Ki-67 proliferative activity,but there are no accurate histological criteria to distinguish benign from malignant forms.Several signaling pathways have been suggested to affect the pathophysiology and behavior of insulinomas;however,our knowledge is limited,urging a further understanding of molecular genetics.Therefore,there is a need for the identification of reliable markers of metastatic disease that could also serve as therapeutic targets in patients with malignant insulinoma.This opinion review reflects on current gaps in diagnostic and clinical aspects related to the malignant behavior of insulinoma.展开更多
Malignant insulinomas are rare neuroendocrine tumors that require management for both symptomatic control and tumor reduction.It is clinically challenging to optimize treatment strategies for refractory malignant insu...Malignant insulinomas are rare neuroendocrine tumors that require management for both symptomatic control and tumor reduction.It is clinically challenging to optimize treatment strategies for refractory malignant insulinoma.We report a case of metastatic grade 3 insulinoma presented with recurrent hypoglycemia in a 23-year-old female with RAD51D p.Q192 germline mutation.During the disease course of 5 years,the tumor has continuously progressed despite locoregional therapy and multiple lines of systemic treatment.However,oxaliplatin-based chemotherapy achieved a partial response,which was maintained for 2 years.The hypoglycemic symptoms were controlled after the treatment response and did not recur.The platinum-based regimen could be a feasible therapeutic strategy for malignant insulinoma.The relationship between germline mutation in the DNA damage repair pathway and treatment response to platinum-based regimens in neuroendocrine tumors warrants further investigation.展开更多
文摘Insulinomas are the most frequent type of functional pancreatic neuroendocrine tumors with a variety of neuroglycopenic and autonomic symptoms and welldefined diagnostic criteria;however,prediction of their clinical behavior and early differentiation between benign and malignant lesions remain a challenge.The comparative studies between benign and malignant cases are limited,suggesting that short clinical history,early hypoglycemia during fasting,high proinsulin,insulin,and C-peptide concentrations raise suspicion of malignancy.Indeed,malignant tumors are larger with higher mitotic count and Ki-67 proliferative activity,but there are no accurate histological criteria to distinguish benign from malignant forms.Several signaling pathways have been suggested to affect the pathophysiology and behavior of insulinomas;however,our knowledge is limited,urging a further understanding of molecular genetics.Therefore,there is a need for the identification of reliable markers of metastatic disease that could also serve as therapeutic targets in patients with malignant insulinoma.This opinion review reflects on current gaps in diagnostic and clinical aspects related to the malignant behavior of insulinoma.
文摘Malignant insulinomas are rare neuroendocrine tumors that require management for both symptomatic control and tumor reduction.It is clinically challenging to optimize treatment strategies for refractory malignant insulinoma.We report a case of metastatic grade 3 insulinoma presented with recurrent hypoglycemia in a 23-year-old female with RAD51D p.Q192 germline mutation.During the disease course of 5 years,the tumor has continuously progressed despite locoregional therapy and multiple lines of systemic treatment.However,oxaliplatin-based chemotherapy achieved a partial response,which was maintained for 2 years.The hypoglycemic symptoms were controlled after the treatment response and did not recur.The platinum-based regimen could be a feasible therapeutic strategy for malignant insulinoma.The relationship between germline mutation in the DNA damage repair pathway and treatment response to platinum-based regimens in neuroendocrine tumors warrants further investigation.
