A Case of Acute Psychosis as Manifestation of Ectopic ACTH Producing Pancreatic Neuroendocrine Tumor

Abstract Introduction: Ectopic adrenocorticotrophic hormone (ACTH) producing Pancreatic Neuroendocrine Tumor (p-NET) are extremely rare with incidence of 1.2% and confer a poor prognosis (5-year survival <20%). Case Report: 31-year old female diabetes mellitus type 2 (DM-2) hypertension (HTN) was found to be severely hypokalemic on routine labs sent the Emergency Department for potassium replacement. Vitals; BP:139/87 mmHg, HR:108 bpm, RR:18, T:37°C saturating 98% ambient air. Examination revealed cushingoid features; hyperpigmented face, bilateral upper extremity pigmented papules, proximal muscle weakness, acanthosis nigricans, violaceous abdominal striae, truncal obesity, ecchymoses, lower edema, memory impairment anxiety. Initial labs; BG: 289 mg/dL, HbA1c: 9.5%, K: mmol/L (N 3.5-5.2), WBC: 12.5 k/μL 4-10.8), Cortisol >150 mcg/dL 5-23), TSH: 0.325 μIU/mL 0.45-4.5), FT4: 0.69 ng/dL 0.82-1.77). Diagnosis Cushing’s syndrome made based elevated cortisol level confirmed 24-hour urinary free cortisol: 13,294 μg (N<45), midnight salivary >15 ug/dL 0.09-1.5) 1 mg dexamethasone suppression test (Cortisol mcg/dL, ACTH: 621 pg/ml). Additional showed Chromogranin A: 717 ng/mL 0-160). She started prophylactic Enoxaparin & Trimethoprim/Sulfamethoxazole treated Spironolactone, Basal/Bolus insulin, Ketoconazole hypercortisolemia. Pituitary MRI negative pituitary adenoma. Abdominal/Pelvic CT scan demonstrated solid 5 cm pancreatic head mass innumerable haptic metastases consistent metastatic p-NET which PET-CT Ga68 Dotatate scan. Liver biopsy positive well differentiated ki-67 <5% ACTH staining. Hospital course complicated by fluctuating levels intermittent psychotic manifestations despite up-titration Ketoconazole, addition Octreotide as anti-psychotics. later developed transaminitis maximum dose thus underwent laparoscopic total adrenalectomy treatment her Post-operatively, she Hydrocortisone Fludrocortisone Spironolactone discontinued. transitioned monthly Lanreotide Capecitabine Temozolomide adequate therapeutic response subsequent down trending 188 228 pg/ml levels. Currently, is doing significant improvement DM-2, HTN, psychiatric symptoms overall clinical status. Conclusions: Symptoms ectopic may delayed, however given aggressive nature p-NET, timely diagnosis, prophylaxis individualized approach crucial in achieving favorable prognosis.