Abstract 12934: Refractory Hypercholesterolemia Induced by Treatment of Adrenocortical Carcinoma Demonstrates a Significant Challenge in Cardiovascular Disease Risk Management

• Published in: Circulation (New York, N.Y.) N.Y.), 2022-11, Vol.146 (Suppl_1), p.A12934-A12934
• Main Authors: Wilbur, Jameson G, James, Amy, Wyne, Kathleen L
• Format: Article
• Language: English
• ISSN: 0009-7322; 1524-4539
• DOI: 10.1161/circ.146.suppl_1.12934

IntroductionHypercholesterolemia is a significant modifiable risk factor for cardiovascular disease (CVD). Prompt management of high-risk patients presenting with this condition is paramount, particularly for those with other comorbid conditions. Case DescriptionA 37-year-old female with long-standing well-controlled type 1 diabetes (over 30 years), metabolic syndrome, and mildly elevated LDL (128-138 mg/dL) on maximally tolerated statin therapy was diagnosed with adrenocortical carcinoma. She continued statin therapy through multiple different anti-tumor protocols including traditional agents, immunotherapy and mitotane. She returned to diabetes clinic twenty months after the diagnosis, after starting a new anti-tumor protocol following progression of disease despite three different protocols, where lipid profile was drawn showing severe hypercholesterolemia with elevations in total cholesterol (489 mg/dL) and LDL-c (388 mg/dL). Given her risk for CVD, intensive lipid lowering therapy was started by our multidisciplinary treatment team. LDL-c did not attain goal on a PCSK9-inhibitor, nor did it improve with testing a high dose of a different statin. Finally, after testing multiple different agents and combinations over the subsequent twelve months, the LDL-c has achieved goal of < 100 mg/dL on a multi-drug regimen consisting of a PCSK9-inhibitor, statin, ezetimibe, and bempedoic acid. The LDL-c has now been maintained within goal range for six months. DiscussionThis case illustrates the potential for severe refractory hypercholesterolemia induced by the treatment of adrenocortical carcinoma. Little literature exists guiding therapy for these patients. The present case suggests management may require multimodal therapy targeting several mechanisms of lipid metabolism, an escalation from traditional therapy. As survival outcomes for patients with many cancers improve with advances in treatment, management of risk factors for CVD is tantamount for reducing long-term morbidity and mortality. While routine lipid monitoring is not typically performed during cancer treatment, it may well be beneficial. Further research optimizing screening and treatment is warranted.