Adrenocorticotropic hormone-secreting pancreatic neuroendocrine carcinoma with multiple organ infections and widespread thrombosis:A case report

BACKGROUND Ectopic adrenocorticotropic hormone(ACTH)-secreting neuroendocrine tumors are rare diseases.Patients with ACTH-secreting pancreatic neuroendocrine carcinomas have a poor prognosis.Infections and coagulopathies have been reported as the cause of death.However,detailed clinical descriptions of the morbid complications of ACTH-secreting neuroendocrine carcinomas have not been reported.CASE SUMMARY A 78-year-old Japanese woman consulted a medical center due to systemic edema and epigastric discomfort.Laboratory analysis revealed hypercortisolemia with increased ACTH secretion without diurnal variation in serum cortisol level.An enhanced computed tomography(CT)scan revealed a 3-cm tumor in the pancreatic head.The cytological material from endoscopic ultrasound-guided fine-needle aspiration was compatible with ACTHsecreting pancreatic neuroendocrine carcinoma.The Ki-67 index was 40%.She was transferred to Mie University Hospital for surgical treatment.The patient was diagnosed with urinary tract infection,cytomegalovirus hepatitis,esophageal candidiasis,pulmonary infiltrates suspicious for Pneumocystis carinii pneumonia,peripheral deep vein thrombosis,pulmonary embolism,and disseminated intravascular coagulation.The multiple organ infections and thromboses responded well to antimicrobial and anticoagulant therapy.Radioisotope studies disclosed a pancreatic tumor and a metastatic lesion in the liver,whereas somatostatin receptor scintigraphy showed negative findings,suggesting the primary and metastatic tumors were poorly differentiated.A CT scan before admission showed no metastatic liver lesion,suggesting that the pancreatic tumor was rapidly progressing.Instead of surgery,antitumor chemotherapy was indicated.The patient was transferred to another hospital to initiate chemotherapy.However,she died four months later due to the rapidly progressive tumor.CONCLUSION ACTH-secreting pancreatic neuroendocrine neoplasm is a rare disease with a very poor prognosis.The clinical course and acute complications of the tumor remain unreported.Here we report the clinical course of a rapidly progressive case of ACTH-secreting pancreatic neuroendocrine tumor that developed infectious complications due to many types of pathogens in multiple organs,widespread thromboses,pulmonary embolism,and disseminated intravascular coagulation.

Sodium-glucose co-transporter-2 inhibitor-associated euglycemic diabetic ketoacidosis that prompted the diagnosis of fulminant type-1 diabetes:A case report

Fulminant type 1 diabetes mellitus(FT1DM)is a subtype of type 1 diabetes mellitus characterized by an abrupt onset and a rapid and complete functional loss of isletβcells.It is a very rare disease generally associated with ketoacidosis and the absence of circulating pancreatic islet-related autoantibodies.Diabetic ketoacidosis with normal blood glucose levels has been reported during sodiumglucose co-transporter 2(SGLT2)inhibitor therapy.CASE SUMMARY The patient was a 43-year-old woman that consulted a medical practitioner for malaise,thirst,and vomiting.Blood analysis showed high blood glucose levels(428 mg/dL),a mild increase of hemoglobin A1c(6.6%),and increased ketone bodies in urine.The patient was diagnosed with type 2 diabetes mellitus.The patient was initially treated with insulin,which was subsequently changed to an oral SGLT2 inhibitor.Antibodies to glutamic acid decarboxylase were negative.Four days after receiving oral SGLT2 inhibitor,she consulted at Mie University Hospital,complaining of fatigue and vomiting.Laboratory analysis revealed diabetic ketoacidosis with almost normal blood glucose levels.The endogenous insulin secretion was markedly low,and the serum levels of islet-related autoantibodies were undetectable.We made the diagnosis of FT1DM with concurrent SGLT2 inhibitor-associated euglycemic diabetic ketoacidosis.The patient's general condition improved after therapy with intravenous insulin and withdrawal of oral medication.She was discharged on day 14 with an indication of multiple daily insulin therapy.CONCLUSION This patient is a rare case of FT1DM that developed SGLT2 inhibitor-associated diabetic ketoacidosis with almost normal blood glucose levels.This case report underscores the importance of considering the diagnosis of FT1DM in patients with negative circulating autoantibodies and a history of hyperglycemia that subsequently develop euglycemic diabetic ketoacidosis following treatment with a SGLT2 inhibitor.