Malignant nonfunctioning paraganglioma of the retroperitoneum producing renovascular hypertension

Malignant nonchromaffin paraganglioma (chemodectoma) is an unusual tumor arising from chemoreceptor cells derived from the neural crest. Although these tumors generally occur in the head and neck, where the term "carotid body tumor" applies, more than 20 cases of retroperitoneal origin have been described. This case report presents the clinical, radiographic, and pathologic features of a chemodectoma arising in the retroperitoneal area that produced severe hypertension by unilateral real vascular compression. The patient underwent exploratory laparotomy, and a left nephrectomy was done. Follow-up examination 1 year after surgery disclosed no clinical evidence of disease, and he had normal blood pressures without medication.