FRI199 Severe Hypertriglyceridemia And Acute Pancreatitis In A Patient With Adrenocortical Carcinoma (ACC) Taking Mitotane
Authors: Cortez Briana Noel , Varghese Diana Grace , Naimian Amirkia , Mallorson Rosa , Magee Tamika , Kelley Sarah , Roper Nitin , Fojo Antonio T , Del Rivero Jaydira 
Published: October 2023

Disclosure: B.N. Cortez: None. D.G. Varghese: None. A. Naimian: None. R. Mallorson: None. T. Magee: None. S. Kelley: None. N. Roper: None. A.T. Fojo: None. J. Del Rivero: None.

Background: Adrenocortical carcinoma (ACC) is a rare tumor with an incidence of 0.7-2 per million people per year, and a poor prognosis with a five-year survival &lt;35%. Many patients present with steroid hormone excess (glucocorticoids, mineralocorticoids, androgens). Treatment of choice for a localized primary or recurrence is radical surgical resection, which offers the best chance for prolonged recurrence-free survival. However, patients with recurrent or metastatic disease are infrequently curable by surgery alone. Chemotherapy options have limited benefit, though platinum-based therapies have response rates of 25-30%. Mitotane has been used for the treatment of advanced ACC since the 1960s and is generally recommended to reduce risk of future recurrence. Clinical case: A 36-year-old female of Ashkenazi Jewish ancestry presented with a prior history of well-differentiated, grade 1 pancreatic neuroendocrine tumor (NET) fully resected with no evidence of recurrence. On follow up MRI 7 months after resection, she was found to have a 3.8 x 2.5 x 3.3 cm bilobed left adrenal mass. Laparoscopic biopsy was initially reported as NET, however, a second opinion was reported as ACC. She subsequently had laparoscopic surgery with intraoperative tumor spillage and surgical pathology was consistent with high grade ACC, Ki67 of 15-25% with invasion through the adrenal capsule. Germline genetic testing showed no variants of concern. Post-adrenalectomy, the patient’s starting dose was 1g mitotane each day with replacement hydrocortisone. When on 2.5 grams daily, her symptoms were mild diarrhea, fatigue and occasional headaches. One month after starting mitotane, she developed extreme abdominal pain, nausea and vomiting and presented to the ED. Computed tomography at the time revealed no evidence of disease (5 months after resection). Laboratory results were significant for mild leukocytosis, as well as elevated lipase (510 U/L, 0-160), cholesterol (451 mg/dL, 125-200), triglycerides (&gt;10,000 mg/dL, &lt;150), and LDL (168 mg/dL, &lt;100), with a lactic acid of 2.6 mmol/L (&lt;2) consistent with acute pancreatitis likely due to hypertriglyceridemia. Microbiology positive for parainfluenza virus. Her condition worsened and she subsequently developed septic shock, acute renal failure, for which she received hemodialysis, and multiorgan failure. On her ninth day of admission, she succumbed to disease. Conclusion: While some studies show hypertriglyceridemia secondary to mitotane, others only show elevated cholesterol at 6 and 3 months, respectively. We describe an ACC patient with a history of fatty liver disease and mixed hyperlipidemia who developed severe hypertriglyceridemia and acute pancreatitis leading to multiorgan failure about one month after starting mitotane. This case highlights hypertriglyceridemia as a serious complication of mitotane in a patient with preexisting dyslipidemia.

Presentation: Friday, June 16, 2023