摘要
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.