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

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.