SUN-LB37 Atypical Presentation of Primary Adrenal Angiosarcoma With Cushing’s Syndrome - a Case Report

Angiosarcoma is a rare aggressive malignancy originating from the endothelium of blood and lymphatic vessels, accounting for 1% of all tissue sarcomas. These tumors usually invade skin and soft tissue, however they may also invade visceral organs and bones as metastatic disease. Approximately 50 cases have been reported in the literature describing primary adrenal angiosarcomas (PAA). This case represents the first report to describe concomitant Cushing’s syndrome in the setting of epithelioid PAA. A 38-year-old female with pre-diabetes mellitus and hyperlipidemia presented to the emergency department complaining of cough with bloody sputum. Physical exam was remarkable for bilateral lower extremity edema; no Cushing’s features appreciated. Bilateral subsegmental and lobar pulmonary emboli (PE) along with lytic lesions on the ribs were found on Computed Tomography (CT) PE protocol. Chest CT Angiography to prepare for arterial embolization to treat hemoptysis, demonstrated a large heterogeneous enhancing mass arising from the adrenal gland. MRI with adrenal protocol to evaluate incidentaloma revealed an indeterminate 5.2 x 5.6 x 5.2 cm right adrenal mass with central necrosis.Biochemical evaluation revealed a nonsupressible 1mg dexamethasone suppression test (3.3 ug/dL, ref: <1.8 ug/dL), slightly elevated urine metanephrines (191 ug/d, ref: 39 -143 ug/d) and normal plasma metanephrines (0.16 nmol/L, ref: 0.0 - 0.49 nmol/L). Given the biochemical evidence of mild hypercortisolism and uncertainty of etiology of other CT findings, the decision was made to pursue adrenalectomy to aid in the presumed oncologic diagnosis. She underwent successful adrenalectomy, however the postoperative course was complicated by hypotension resistant to fluids. ACTH stimulation test revealed a baseline cortisol <1.0 (ref: 0.0-9.0 ug/dL) with an ACTH of 8 pg/mL (ref: 6 to 58pg/mL), and 60 minute post stimulation cortisol level was 5.5 ug/dL (ref: >18.0 ug/dL). She was started on hydrocortisone replacement therapy, with clinical improvement in hypotension and was eventually tapered off steroids completely.Surgical pathology identified positive staining for CD31, CD34, factor 8, CAM 5.2, and Vimentin consistent with epithelioid angiosarcoma with lymphovascular invasion. There was additionally evidence of adrenocortical hyperplasia on the tissue resected. She established care with oncology and is currently completing 8 cycles of paclitaxel for metastatic disease to bones and lungs. Our patient’s pathology results, and postoperative course suggest the presence of concomitant non-ACTH dependent Cushing’s syndrome associated with epithelioid PAA. This case highlights the importance of thorough biochemical testing before adrenalectomy in the setting of PAA given the possibility of Cushing’s syndrome, which could contribute to post-surgical complications, and prolonged hospital stays.