Bilateral pheochromocytomas with locally advanced right adrenal tumor extending into the inferior vena cava in a suspected Von Hippel–Lindau syndrome: A case report

We report a case of bilateral pheochromocytomas in a 45-year-old man with cutaneous findings suggestive of a hereditary disease. Imaging revealed a large right adrenal tumor extending into the inferior vena cava (IVC) and a smaller contralateral lesion. The patient underwent right adrenalectomy with IVC thrombectomy, while the left lesion was managed conservatively. Histopathology confirmed pheochromocytoma with extensive IVC tumor thrombus and no distant metastases. A residual IVC thrombus was treated with anticoagulation and remained stable on follow-up imaging. This case highlights the complexity of managing hereditary pheochromocytomas with vascular invasion.