A case of juxtaglomerular cell tumor with an unusual clinical presentation

Juxtaglomerular cell tumor (JGCT) is a rare tumor. It is a renin-secreting neoplasm that can cause secondary hypertension. It generally affects adolescents and young adults. Patients typically present with chief complaint of headache leading to a diagnosis of severe hypertension. Herein we report a case of JGCT in a 19-year-old Asian female who initially presented with neurological deficits, and was subsequently found to be hypertensive. She had elevated levels of renin and aldosterone with hypokalemia, and responded to renin inhibitors and an aldosterone antagonist. CT scan demonstrated a 2.2 cm left renal tumor. A partial nephrectomy was performed. Histology showed a well-circumscribed tumor consisting of sheets of polygonal to ovoid cells with eosinophilic cytoplasm and indistinct cell borders. The neoplastic cells are positive for CD34 and synaptophysin. CD117 and tryptase highlight the scattered mast cells within the tumor. After surgical removal of the tumor, the patient's blood pressure is normal. We also reviewed the literature and discussed the differential diagnoses.