“The Way to a Man’s Heart” - Adrenocortical Carcinoma With Vascular Invasion Into the Right Atrium and Ventricle

Abstract

Abstract Background: Adrenocortical carcinoma (ACC) is a rare aggressive malignant neoplasm which may present with intravascular extension into the inferior vena cava (IVC) and rarely into the right atrium (RA). Clinical Case: 62-year-old male with no prior known significant medical history presented to ED with 2-day history of mild hematuria with 3-week history of headache. Vital signs were normal other than blood pressure of 198/88 while physical exam was unremarkable. Headache subsided and blood pressure improved to 130/60 range after IV labetalol administration. CT abdomen and pelvis with contrast revealed a large right suprarenal mass extending into the right hepatic vein, IVC, and RA. The right adrenal gland was not visualized while the left adrenal gland and bilateral kidneys were normal. MRI chest, heart and abdomen with contrast showed heterogeneously enhancing lobulated right adrenal mass measuring 11.4 x 11 x 14 cm (AP, transverse, CC, respectively) with extensive tumor thrombus invading the right hepatic vein, IVC, RA and notably protruding into the right ventricle (RV) through the tricuspid valve during diastole. Technitium-99m MDP whole body scan did not show any uptake suspicious for metastases. Pre-op lab assessment showed mildly abnormal 1 mg dexamethasone suppression test but no evidence of ACTH suppression, elevated catecholamines or excess adrenal steroidogenesis. He underwent combined cardiothoracic and abdominal surgery on cardiopulmonary bypass with resection of adrenal mass, removal of thrombus from IVC, RA, RV and patch angioplasty of IVC with bovine pericardium. Pathology report was consistent with ACC (AJCC stage III). On 1 month postoperative follow-up, patient is clinically doing well with plans to start mitotane with addition of etoposide/doxorubicin/cisplatin (EDP) chemotherapy. Conclusion: ACC is a rare, highly aggressive malignancy which may produce extensive intravascular invasion. It may rarely extend to the RA and even rarer into the RV; with 42 and 1 reported cases, respectively. No study has conclusively found that vascular extension of ACC is a poor prognostic factor, hence surgical management is the primary strategy including cases with RA/RV involvement. There is lack of data and consensus regarding adjuvant or palliative medical therapy. However, in phase II trials combination of EDP chemotherapy and mitotane have shown response rates ranging from 11% to 54%. Reference: Alghulayqah, Abdulaziz, et al. “Long-term recurrence-free survival of adrenocortical cancer extending into the inferior vena cava and right atrium: Case report and literature review.” Medicine 96.18 (2017).