Polycythemia Rubra Vera and Sporadic Bilateral Renal Angiomyolipomas: A Case Report

Polycythemia rubra vera (PRV) is a clonal myeloproliferative neoplasm characterized by autonomous production of erythrocytes, neutrophils, and platelets. Angiomyolipomas (AMLs) are benign renal perivascular epithelioid cell neoplasms of which approximately 80% are sporadic. Here, we report synchronous diagnoses of PRV and asymptomatic sporadic bilateral renal AMLs in a 71-year-old woman. We describe her treatment with phlebotomy and hydroxyurea for PRV and surveillance for renal AMLs. We compare the features and treatment of the present case with those of two previously reported women who also had PRV and sporadic renal AMLs. Finally, we discuss the management and acquired genetic basis of both neoplasms.