LAUSR

Juxtaglomerular cell tumor (JGCT) is a rare renal tumor with a predominantly benign clinical course. It affects young adults, who often present with hypertension, hypokalemia, and hyperaldosteronism. The tumor cells are round to spindle-shaped with occasional mild to moderate atypia, but mitotic figures are usually absent. Surgical resection is the treatment of choice. Typically, the blood pressure and renin levels normalize after removal of the tumor. Rare cases of metastatic and recurrent JGCT have been reported including cases with vascular invasion. These cases typically occur in older adults and present with larger tumor size (9-15 cm). We report a case of JGCT, 5.5 cm in greatest dimension, with atypical pathological features including invasion of the renal vein, lymphovascular invasion, and significant pleomorphism with rhabdoid morphology, along with a brief review of the literature.