ERYTHROCYTOSIS COMPLICATED BY MULTIPLE PARAGANGLIOMA

We report the case of a 22-year-old woman with onset of erythrocytosis at the age of 9 years. Endocrinological and radiological examinations revealed an elevated catecholamine level and the presence of multiple abdominal tumors. After the removal of the tumors, the catecholamine level normalized, whereas erythropoietin remained at the same level an d erythrocytosis persisted. The tumor lysate contained considerable am ounts of catecholamine but no erythropoietin. Moreover, no erythropoie tin mRNA was detected in the tumor by in situ hybridization. These dat a suggest that this paraganglioma did not produce erythropoietin. A re view of the literature showed the existence of patients with early-ons et erythrocytosis complicated with paraganglioma, whose erythrocytosis was not relieved even after the resection of paraganglioma.