Clinical features of renal epithelioid angiomyolipoma

Abstract

Objective To explore the pathological and clinical features of renal epithelioid angiomyolipoma (EAML). Methods From February 2001 to June 2010,4 cases of renal epithelioed angiomyolipoma (EAML) diagnosed in our unit were reviewed on their clinical and histopathological feathers retrospectively.The prognosis of renal EAML were evaluated according to the immunohistochemical findings.Of the 4 cases,the first case had a history of nodular sclerosis and presented a sign of hemorrage caused by the rupture of the tumor.The second case also showed a sign of hemorrage complicated with hemorrhagic shock.The third case had no symptom,but suffered from clear cell carinoma concomitantly on the other kidney.The fourth case came for consultation for his bilateral angiomyolipoma.All the 4 cases had fat composition founded in their CT scans. Results The first case was taken biopsy on the tumor of her right kidney,and lost of follow-up 6 months later.The second case undertaken radical nephrectomy,and no tumor recurrence detected after 10 months follow-up.The third case undertaken right nephron-sparing nephrectomy,and left nephron-sparing nephrectomy 6 months later.No recurrence detected during the follow-up 8 months later.Bilateral resection of the angiomyolipoma was performed on the fourth case,and a malignancy on the left kidney was deteceded 6 years later,then a radical nephrectomy was performed.But liver metastasis was founded half a year later during the follow-up.Immunohistochemcial staining of these tumors showed positive HMB45 and Melan-A but negative CK and EMA. Conclusions Renal EMAL has malignant potentiality,and the aggressiveness of it makes the close follow-up compulsive.Immunohistochemcial study is useful for diagnosis and differential diagnosis,even helpful to assess the prognosis. Key words: Renal epithelioid angiomyolipoma; Malignancy; Tuberous sclerosis