an Aggressive Adrenocortical Ccrcinoma in a Young Man Presenting with Abdominal Pain

Presentation Number: SAT 385
Date of Presentation: April 1st, 2017

Romina Lomonaco* and Antonella Bianchi
University of Florida, Gainesville, FL


Background: Adrenocortical carcinoma (ACC) is a rare but highly aggressive malignancy. It has an incidence of 0.5-2 cases per million per year. Many patients have no symptoms until the tumor starts causing mass effect. About 50% of these tumors are hormonally active, being hypercortisolism the most common presentation. The majority of the patients have already metastatic disease at the time of diagnosis, with a survival rate for stage 4 disease of 7-10% at 5 years.

Clinical case: A 19 year-old man presented to the ER complaining of abdominal pain and nausea. Initial CT abdomen/pelvis showed a large right adrenal mass measuring 11x10x16cm with calcifications. Initial endocrine work up was remarkable for slightly elevated plasma and urinary normetanephrines, 1.20 nmol/L (0-0.89) and 673 μg/d (95-379) respectively, with normal metanephrines. Morning baseline cortisol was 22 mcg/dL (7.0-22.0) with ACTH of 31 pg/mL (7-69). A 1mg-dexamethasone suppression test revealed a morning cortisol of 20.2 mcg/dL (dexamethasone level of 427 ng/dL). PRA and aldosterone, potassium, total testosterone and SHBG were within normal limits. DHEA-S value was highly elevated at 1168 μg/dL (88-483). Patient also had normal TSH, FT4, calcium, iPTH, and creatinine. Calcitonin and chromogranin A were negative. Repeat CT abdomen/pelvis confirmed a large right adrenal mass with invasion of the inferior vena cava, hepatic veins and right atrium. CTA chest revealed bilateral pulmonary emboli and innumerable nodules suggestive for metastasis. Echocardiogram revealed a 4.7cm non-mobile mass obstructing the right atrium. Considering the stage of the disease, no surgery was recommended by surgical oncology, cardiothoracic and vascular surgery. CT-guided biopsy of the adrenal mass was performed after alpha and beta blockade. Pathology showed poorly differentiated ACC. Immunohistochemichal profile was positive for MelanA, and focally positive for Synaptophysin and Inhibin, negative for Calretinin and S-100. Patient was initiated on adjuvant mitotane monotherapy. After 1 week of mitotane, DHEA-S level went down to 166 μg/dL. Cortisol levels also decreased due to the adrenolytic effect of mitotane. Patient was initiated on glucocorticoid replacement with 30mg daily. PRA was in the normal range and there was no need for mineralocorticoid replacement. Palliative chemotherapy with cisplatin/etoposide/doxorubincin was also offered to the patient but was decilined.

Conclusion: ACC is an aggressive, rare and heterogeneous tumor with poor prognosis, particularly if it occurs in children/young adults. It is essential to differentiate it from benign adenomas by correlating with clinical, biochemical, imaging and histological features, as the outcome can vary greatly. Also because of the increased incidence of genetic mutations in this population with ACC, genetic testing for TP53 germline mutation should be considered.


Nothing to Disclose: RL, AB