MON-LB037 Adrenocortical Carcinoma: A Case Report

Abstract

Introduction:Unilateral adrenal tumors are relatively common; adrenal incidentaloma has a prevalence of 4% on CT imaging. These masses are classified by their functional and malignant potential. We present here a case of adrenocortical carcinoma (ACC), a rare and often aggressive tumor that accounts for 2% of all adrenal incidentalomas. ACC has a worldwide incidence of disease of 0.5-2 cases per million population per year. While some instances of ACC are associated with hereditary conditions, most cases are sporadic mutations.Case:A 44-year-old male with a past medical history of hypertension, polysubstance use, and anasarca presented with a one-year onset of abdominal swelling. This was associated with a 16 pound weight loss over the prior 4 months and bilateral lower extremity swelling for the previous 3 weeks. Of note the patient had been detained in an adult corrections institution for over 5 years. Review of systems was negative for night sweats, nausea, vomiting, headaches, confusion, or palpitations. CT of the abdomen demonstrated a large mass involving the left adrenal gland measuring 28 x 27 x 31 cm, with punctate and delayed calcifications. Also noted were numerous bilateral lung nodules, and multiple liver lesions. Bilateral lower extremity ultrasound revealed a DVT involving the left gastrocnemius vein. A heparin drip was initiated and the patient was transitioned to Xarelto. CT scan of the chest demonstrated gastroesophageal junction lymphadenopathy, right hilar lymphadenopathy, numerous lung metastases and a prominent lesion in the left lower lung. Laboratory studies included both a low and high dexamethasone suppression test demonstrating a cortisol level 24 mcg/dL, metanephrines < 0.2 nmol/L, low testosterone 2.1 ng/dL (free) and 54 ng/dL (total), and DHEA 7.1 ng/mlL. Aldosterone <4.0 ng/dL and ACTH < 5 pg/mL. Biopsy for the adrenal mas was consistent with cortical neoplasm. Surgery recommend against debulking due to wide-spread metastases. Oncology recommended chemotherapy; a regimen of mitotane, etoposide, doxorubicin, and cisplatin, with glucocorticoid replacement in 28-day cycles x 6 through venous port.Conclusion:Adrenocortical carcinoma is a rare, aggressive tumor that often secretes excess cortisol and/or androgens. In the case presented a large advanced stage ACC was nonfunctional. First-line therapy for ACC includes resection with debulking surgery, radiation therapy, and/or chemotherapy. 5-year survival rates range 62-82% for those with disease confined to the adrenal gland and 13% for tumors associated with distant metastases. Repeat CT scans for our patient demonstrated stable appearance after four cycles of chemotherapy, thus indicating this treatment regimen may be beneficial for such an aggressive disease.