Renal hilar paraganglioma: A case report

Paragangliomas are extra-adrenal pheochromocytomas that derive from chromaffin cells and arise along the sympathetic paraganglia in the body. In the majority of cases, they are secretory tumors and most commonly present with palpitations. Plasma metanephrines are the standard screening tests for making the diagnosis which is confirmed by pathology. Imaging plays a very important role in establishing the diagnosis. However, there is no specific feature on imaging for paragangliomas; the vascularity of the tumor should show as hyper-enhancing lesions but this is not always the case. The diagnostic value of PET is yet a matter of debate. We present a very rare case of a paraganglioma arising at the renal hilum, splaying the renal artery and vein and causing vascular compromise to the left kidney. The patient presented with an atypical presentation of unrelenting fever that was followed by acute colicky pain. Based on imaging and blood metanephrine levels, the diagnosis of paraganglioma was made. Resection of the tumor was achieved and the patient is now asymptomatic.