Angiomyolipoma pathophysiology: Difference between revisions
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==Pathophysiology== | ==Pathophysiology== | ||
Since all three components of an angiomyolipoma (vascular cells, immature smooth muscle cells and fat cells) contain a [[Two-hit hypothesis|"second hit" mutation]], they are believed to have derived from a common [[progenitor cell]] that suffered the common second hit mutation. [[Angiomyolipoma]]s 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. | Since all three components of an angiomyolipoma (vascular cells, immature smooth muscle cells and fat cells) contain a [[Two-hit hypothesis|"second hit" mutation]], they are believed to have derived from a common [[progenitor cell]] that suffered the common second hit mutation. [[Angiomyolipoma]]s 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=== | ===Microscopic Pathology=== | ||
[[Image:Renal angiomyolipoma (1).jpg|thumb|left|350px|1. Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. H & E stain.]] | |||
[[ | |||
Image:Renal angiomyolipoma (1).jpg|thumb|left|350px|1. Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. H & E stain.]] | |||
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Revision as of 15:22, 21 September 2015
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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.]]
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