Juxtaglomerular cell tumor is a rare renal neoplasm with fewer than 100 cases reported in literature, of which 7 cases were diagnosed during pregnancy. This tumor is generally seen in young adults with female preponderance. Patients generally present with hypertension, hyperaldosteronism, and hypokalemia secondary to renin secretion by the tumor cells. When the tumor occurs during pregnancy, the hypertension is often presumed to be pregnancy-induced hypertension and results in fetal loss. We report the case of a 21-year-old woman who presented with hypertension in the first trimester of pregnancy, which was attributed to and treated as pregnancy-induced hypertension. Routine ultrasonography of abdomen in the fourth month revealed a right renal mass. Patient underwent preterm delivery in the seventh month, and the infant did not survive, and subsequently after 1 month, a partial nephrectomy was performed. The diagnosis of juxtaglomerular cell tumor was confirmed by light microscopy. The tumor was positive for CD34 and vimentin. Electron microscopy showed the characteristic rhomboid renin granules in the cytoplasm of the tumor cells.
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