Rare case of left adrenal cortical carcinoma with level 3 inferior vena cava thrombus via adrenal vein

Abstract

Introduction: Adrenocortical carcinoma (ACC) is a rare malignancy with an incidence of 0.7–2.0 cases/million habitants/year. It shows a bimodal pattern of age distribution with a higher incidence in the first decade and then between 40 and 50 years. Women are most frequently affected (55–60%). Observation: A 21-year-old male, incidentally detected with left supra renal tumor invading the left renal vein and the thrombus extending into the supra diaphragmatic IVC, underwent laparotomy with simultaneous median sternotomy on total cardiac bypass for removal of IVC tumor thrombus and radical excision of the tumour with left nephrectomy and spleenectomy. The histopathology report came to be adrenal cortical carcinoma with no renal parenchymal invasion and the immuno-histochemistry showing it to be positive for synaptophysin, inhibin and KI-67 (15%) while negative for chromogranin, pan cytokeratin and CD-10 receptors. Patient then received 3 weekly 6 cycles of adriamycin and cisplatin chemotherapy. Bone scan and CECT abdomen and thorax done in the follow up after two years were normal. There are not many cases of adrenal carcinoma reported in the literature, but it has been seen that it rarely shows venous thrombosis in the IVC. In all the reported cases radical surgery is the preferred treatment option, even in the tumors extending to the right atrium. The unique feature in our case is that the tumor has extended to the supra diaphragmatic IVC via the left adrenal vein, without the renal parenchyma involvement. Conclusion: The intravascular extension of the adrenal carcinoma is rare but its presence alone is not a contraindication to radical surgery, as it is the best hope for prolonged survival.

African Journal of Urology (2017) 23, 201–203