MON-344 A Large Adrenal Mass with Neuroendocrine Features Presenting as Pulmonary Embolism: A Case Report

Abstract

Background: Adrenocortical Carcinoma (ACC) is a rare disease that has a bimodal distribution. 60% of ACC presents with symptoms and signs of adrenal steroid hormone excess [1] while non-functional ACCs present less conspicuously with abdominal mass or other nonspecific symptoms making diagnosis challenging. The usual sites of metastasis are the lung (71%), lymph node (68%), liver (42%), and bone (26%) [1]. According to literature, there has been few reported cases of ACC extending to the inferior vena cava and right atrium presenting with pulmonary embolism. Clinical Case: Patient is a 38 year old male admitted due to dyspnea, BP elevation and weight loss. Initial CXR was done showing canon ball lesions. On work up, Chest CT scan revealed pulmonary metastasis and pulmonary embolism. Abdominal CT scan showed a large right suprarenal mass with tumor infiltrating the inferior vena cava, hepatic and portal veins. 2 d echo revealed a large pedunculated mass attached to the free wall of the right atrium. The patient was started on anticoagulation. Diagnostic work up for adrenal function was done. Plasma renin and aldosterone in supine position were 3.30ng/mL/hr (reference range 0.3-1.9), and 23.28 ng/dL (reference range: 3.78-23.3) respectively with an ARR 7. 8 am cortisol was 12.30 ug/dL (reference range: 3.7-19.4ug/dL), 24 hour urine metanephrine 0.43 (reference range: 0-1) and plasma free metanephrine 36.75 pg/mL (reference range <65pg/mL) were all normal. Chromogranin A was normal at 90.71 ng/mL (reference range: <100ng/mL) however, DHEAS was 3x elevated at 1,406.00 ug/dL (Reference Range: 139.70–484.40 ug/dL). The patient underwent FNAB of the lung nodules revealing a malignant neoplasm with neuroendocrine features. Since the etiology of the adrenal mass was still uncertain, a functional imaging with 68Ga-DOTATATE PET-CT scan was done. However, the large right suprarenal mass was mostly necrotic with a small 68Ga-DOTATATE-avid focus. During admission, patient had sudden onset of chest pain, diaphoresis, hypotension and desaturation. He deteriorated and eventually expired. Post mortem examination revealed adrenocortical carcinoma with neuroendocrine features; hemorrhagic infarction secondary to massive pulmonary tumor embolism. Conclusion: Adrenocortical carcinoma is a rare cancer with varied initial presentation. A rare presentation is pulmonary embolism. In advanced stage diseases, with distant metastases, ACC has a poor prognosis. Multimodality approach with radiotherapy, aggressive surgical resection and mitotane can be offered as treatment options. Reference: 1. Kim, KH, et al. "A case of non-functioning huge adrenocortical carcinoma extending into inferior vena cava and right atrium."