Angiomyolipoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2], Faizan Sheraz, M.D. [3]

Pathophysiology

Since all three components of an angiomyolipoma (vascular cells, immature smooth muscle cells and fat cells) contain a "second hit" mutation, they are believed to have derived from a common progenitor cell that suffered the common second hit mutation. Angiomyolipomas are members of the perivascular epithelioid cells tumour group (PEComas) and are composed of variable amounts of three components; blood vessels (-angio), plump spindle cells (-myo) and adipose tissue (-lipo). Almost all classic angiomyolipomas are benign but they do have the risk of rupture with bleeding or secondary damage/destruction of surrounding structures as they grow.

Variants

There is a special variant called an epithelioid angiomyolipoma, composed of more plump, epithelial looking cells, often with nuclear atypia, that have a described risk of malignant behaviour. This variant, unlike conventional AMLs, may mimic renal cell carcinoma.10 Metastases have also been described 9.

Microscopic Pathology

[[

Image:Renal angiomyolipoma (1).jpg|thumb|left|350px|1. Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. H & E stain.]]

2. Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. The same case as demonstrated in "Image 1". H & E stain.

3. Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. The same case as demonstrated in "Image 1". H & E stain.

4. Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. The same case as demonstrated in "Image 1". HMB-45 immunostain.