(1.07) Paraneoplastic Syndrome in Adrenocortical Carcinoma, The Unexpected Mime.

A 68-year-old female with type 2 diabetes mellitus and prior PE from COVID-19 infection was sent to the ED by her endocrinologist’s office after complaining of fatigue, dizziness, and diaphoresis. With an associated 20 pounds of weight loss and uncontrolled hyperglycemia for 6 months before the visit. Blood glucose was 547 mg/dl on a point-of-care finger stick. In the ED, the patient was tachycardic, she had a round face and central obesity. CT angiography of the chest demonstrated an acute PE with right ventricular strain, pulmonary nodules, and left external iliac vein thrombosis. Adrenal CT scan with IV contrast and subsequent MRI with and without contrast showed a 6.9 x 5.6 x 5.2 cm bilobed adrenal mass. The mass invaded the left renal and splenic veins, the greater curvature of the stomach, and a nonocclusive thrombus in the left renal vein was present. Intravenous (IV) anticoagulation and right pulmonary arterial thrombectomy were done and later complicated by extensive bleeding requiring multiple blood transfusions. A hormonal workup suggested adrenal hypercortisolism. The patient underwent left adrenalectomy, left radical nephrectomy, splenectomy, distal pancreatectomy, and caval thrombectomy with IVC filter placement. IV dexamethasone was initiated for secondary adrenal insufficiency. Pathology confirmed oncocytic adrenal cortical carcinoma with high-grade mitoses and a Ki-67 labeling index of 30%. Post-surgical course was complicated by the formation of a right abdominal wall, liver hematomas, and worsening DVT. Eventually, the patient was discharged home with hospice care. 

Discussion : ACC is a rare malignancy with an incidence of 2/1,000,000 people/year. More than 50% are functional masses, with most of them producing excess cortisol. Diagnoses can be challenging as symptoms will vary depending on the hormone overproduction. In less than 3% of cases, patients can initially present with thrombosis and even more rarely, with pulmonary and intracardiac embolism. DVT in ACC can be caused by increased hypercoagulability from hypercortisolism, direct venous thrombosis, or vascular invasion. Our patient presented with Cushing syndrome and multiple thrombotic events and was also very debilitated and immobile, adding venous stasis as another risk factor. Thrombosis, especially in the inferior vena cava, has been associated with poor prognosis and survival rates. Clinicians should be aware of this rare complication of ACC given its both immediate therapeutic repercussions and prognostic value.