FRI249 New Onset Heart Failure In A Patient With Rapidly Progressive Adrenocortical Carcinoma

Disclosure: J. Peeples: None. S. Shu: None. K.G. Romo: None. J. Tereul: None. B.B. Grissett: None. Background: Adrenocortical carcinomas (ACC) are rare, relatively large (>4cm), lipid-rich, and often aggressive tumors. They may be functional, which typically presents as Cushing’s syndrome and/or virilization, or non-functional, which typically presents as an abdominal mass or incidental finding. They occur in two peak incidences: at the first decade of life and between 40-50 years of age. Over half of adult patients with ACC present with an advanced tumor stage. This case describes a young man with new onset heart failure and adrenal mass with metastases who was found to have biopsy-confirmed ACC. Clinical Case: A 20-year-old man with a history of cigarette smoking and marijuana use, but otherwise in prior good health, presented as a transfer for new onset heart failure in the setting of hypertensive emergency (SBP 260s), and CT findings of left adrenal mass, with multiple lung and liver masses. He noted 2 months of rapidly progressive weight gain, easy fatigability, dyspnea on exertion, and leg swelling. Exam revealed central adiposity with abdominal striae, bilateral clavicular and cervical fat pad, and bilateral lower extremity swelling. Investigations revealed hypercortisolism and ventricular ejection fraction 20-25%. Liver mass biopsy revealed metastatic ACC. Family history was significant for multiple members with early onset hypertension and congestive heart failure, including the death of maternal grandmother at age 40. The patient was initially managed in the ICU with an etomidate drip for adrenal suppression. Cortisol levels appropriately decreased to goal. Additionally, he started IV dexamethasone while inpatient. He was discharged once euvolemic on PO furosemide and follow-up was scheduled with cardiology, oncology, and endocrinology. At discharge, he transitioned to PO hydrocortisone (HC) and was placed on trimethoprim/sulfamethoxazole for PJP prophylaxis, doxazosin, and ketoconazole. He also started mitotane for his cancer treatment, with HC to prevent adrenal insufficiency while on mitotane. Subsequently, he started and failed carboplatin plus etoposide palliative chemotherapy with a continued increase in adrenal mass size. Now, he is on second line pembrolizumab with an ECOG score of 2. Conclusion: ACC is a rare and often aggressive tumor that should be considered in the setting of hypercortisolism and cushingoid features. This is particularly important for patients with a relatively unremarkable history and new onset heart failure with dyspnea on exertion. In these cases, mitotane can be used for hypercortisolism associated with ACC. Unfortunately, these tumors are usually diagnosed when they have already progressed to a late stage, and the prognosis for ACC is poor. Presentation: Friday, June 16, 2023