Polycythemia vera as a presentation of renal angiomyolipoma: a case report.

Journal of Medical Case Reports
Ming-Shyan LinPao-Hsien Chu

Abstract

Angiomyolipoma is a common benign renal tumor composed of thick-walled blood vessels, smooth muscle, and adipose tissue. It may be found incidentally during workup for suspected renal disease. Although angiomyolipoma may present as a palpable, tender renal mass with flank pain and gross or microscopic hematuria, many patients are asymptomatic. Erythrocytosis is an unusual presentation, and malignant transformation may be suspected. This report describes a rare case of a woman diagnosed with renal angiomyolipoma and polycythemia vera. The report discusses the differential diagnosis using erythropoietin, erythropoietin-receptor and Janus kinase 2. A 79-year-old Chinese woman was diagnosed with erythrocytosis according to World Health Organization criteria. An upper left renal pole angiomyolipoma was successfully ablated after multiple phlebotomy treatments. Red cell count immediately returned to normal, but gradually increased after 4 months. Polycythemia vera was finally diagnosed by positive mutation of Janus kinase 2 and negative erythropoietin protein expression. Her clinical symptoms improved with regular phlebotomy and hydroxyurea treatment. Concurrent occurence of angiomyolipoma and polycythemia vera is rare. Polycythemia ...Continue Reading

References

Oct 22, 1998·International Journal of Urology : Official Journal of the Japanese Urological Association·T HanadaY Nomura
Apr 11, 2000·Scandinavian Journal of Urology and Nephrology·S K YipK T Foo
Sep 28, 2002·The Journal of Urology·Caleb P Nelson, Martin G Sanda
Apr 16, 2003·International Journal of Urology : Official Journal of the Japanese Urological Association·Nobuyoshi TakahashiTadashi Suzuki
May 9, 2003·Urology·Albert OngLouis Kavoussi
Sep 21, 2006·International Journal of Urology : Official Journal of the Japanese Urological Association·Luca MorelliBruno Monica
Apr 25, 2007·Hematology·Richard C TurkingtonMary F McMullin

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