Case
53 Diagnosis and discussion |
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Diagnosis: Angiomyolipoma with spontaneous rupture
Renal angiomyolipoma have an incidence of 0.3-3% of renal neoplasm and arise from the mesenchymal elements of the kidney. They are mostly benign tumors composed of varying proportions of mature adipose tissue, smooth muscle and abnormal thick walled blood vessels. Angiomyolipomata have been described in the literature either as hamartomas or choriostomas. As fat and smooth muscle are not normal constituents of the renal parenchyma, angiomyolipoma by definition should be considered as choriostoma; however genetic studies have shown it be a clonal neoplastic proliferation (Heptinstal's Pathology of the kidney, 6th ed, LWW, p. 1516).
Spontaneous rupture of renal angiomyolipoma is rare but is a well-recognized surgical emergency. Resulting hematoma is confined to either the subcapsular or perinephric space.
The present case has a typical microscopic appearance and not ancillary studies were necessary for diagnosis. Even so, HMB45 and smoott muscle actin immunohistochemistry was carried out, demonstrating the characteristic positivity. Other less typical angiomyolipomas, as epitheliod, smooth muscle predominance, can require ancillare suties, manly IHC, por a precise diagnosis.
See the chapter Renal Neoplasm, part 2, of our Tutorial (this chapter is not yet available in English).
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