Case Report: Peri-operative management of ACTH-secreting pancreatic neuroendocrine tumor and major vessel reconstruction

Adrenocorticotrophic hormone (ACTH)-secreting pancreatic-neuroendocrine-tumors are extremely rare.  They present a significant peri-operative management challenge because of the tumor’s extensive vascular involvement as well as excess hormone production resulting in electrolyte and metabolic disturbances.   A 49-year-old male presented for resection of his ACTH-secreting pancreatic-neuroendocrine-tumor involving the pancreatic head and many major vessels. Pre-operative assessments showed increased serum cortisol, low serum ACTH, negative 24-hour urine 5-hydroxyindoleacetic-acid, and no suppression with 1mg dexamethasone. Pre-operatively, he had ectopic Cushing’s syndrome, type two diabetes mellitus, hypokalemia and thrombocytopenia for which he was treated with insulin, intravenous potassium, and platelets. He was given octreotide at the beginning of surgery. The 10-hour procedure involved both general and vascular surgery. There was eight liters of blood loss and the patient required significant transfusions. At the end of surgery, he remained ventilated due to the duration of surgery and amount of blood loss. Post-operatively, he did well, but he developed adrenal insufficiency secondary to removal of his ACTH-secreting tumor and was discharged home on hydrocortisone.   This case highlights the role of anesthesiologists as the peri-operative physician in maintaining homeostasis in these complex patients. It also shows the importance of pre-operative tumor characterization, carcinoid crisis prophylaxis, and a multi-disciplinary approach to the management of these rare tumors.