Angiomyolipoma pathophysiology: Difference between revisions
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{{Angiomyolipoma}} | {{Angiomyolipoma}} | ||
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==Overview== | ==Overview== | ||
Revision as of 10:50, 11 September 2018
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Angiomyolipoma Microchapters |
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Diagnosis |
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Treatment |
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Case Studies |
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Angiomyolipoma pathophysiology On the Web |
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American Roentgen Ray Society Images of Angiomyolipoma pathophysiology |
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Risk calculators and risk factors for Angiomyolipoma pathophysiology |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2] Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [3], Faizan Sheraz, M.D. [4], Rekha, M.D.
Overview
On gross pathology, well circumscribed and uniform yellow mass is characteristic finding of angiomyolipoma. The components of an angiomyolipoma include vascular cells, immature smooth muscle cells and fat cells. Angiomyolipoma is caused by a defect in TSC1 gene. Diseases associated with angiomyolipoma include von Hippel-Lindau syndrome and tuberous sclerosis. Cytologic features of angiomyolipoma include round nuclei and bland chromatin. Immunohistochemistry markers of angiomyolipoma include positive melanocytic markers.
Pathophysiology
Three components of an angiomyolipoma include:
- Vascular cells
- Immature smooth muscle cells
- Fat cells
Angiomyolipoma is derived from a common progenitor cell that suffered the common second hit mutation. Angiomyolipomas are members of the perivascular epithelioid cells tumor group (PEComas) and are composed of variable amounts of these three components.
Genetics
Angiomyolipoma is caused by a defect in:
Associated Conditions
Diseases associated with angiomyolipoma include:
Gross Pathology
On gross pathology, well circumscribed and uniform yellow mass are characteristic findings of angiomyolipoma.
Microscopic Pathology
Microscopic features of angiomyolipoma:[2]
- Smooth muscles
- Adipose tissue
- Abundant blood vessels
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![Histopathologic slide of renal angiomyolipoma. Nephrectomy specimen. H & E stain.[3]](/images/a/a3/Renal_angiomyolipoma_%281%29.jpg)
Histopathologic slide of renal angiomyolipoma. Nephrectomy specimen. H & E stain.[3]
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![Histopathologic slide of renal angiomyolipoma. Nephrectomy specimen. H & E stain.[3]](/images/b/be/Renal_angiomyolipoma_%282%29.jpg)
Histopathologic slide of renal angiomyolipoma. Nephrectomy specimen. H & E stain.[3]
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![Histopathologic slide of renal angiomyolipoma. Nephrectomy specimen. H & E stain.[3]](/images/d/d2/Renal_angiomyolipoma_%283%29.jpg)
Histopathologic slide of renal angiomyolipoma. Nephrectomy specimen. H & E stain.[3]
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![Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. HMB-45 immunostain.[3]](/images/0/07/Renal_angiomyolipoma_%284%29_HMB-45_immunostain.JPG)
Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. HMB-45 immunostain.[3]
![Histopathologic slide of renal angiomyolipoma. Nephrectomy specimen. H & E stain.[3]](/index.php/File:Renal_angiomyolipoma_(1).jpg)
![Histopathologic slide of renal angiomyolipoma. Nephrectomy specimen. H & E stain.[3]](/index.php/File:Renal_angiomyolipoma_(2).jpg)
![Histopathologic slide of renal angiomyolipoma. Nephrectomy specimen. H & E stain.[3]](/index.php/File:Renal_angiomyolipoma_(3).jpg)
![Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. HMB-45 immunostain.[3]](/index.php/File:Renal_angiomyolipoma_(4)_HMB-45_immunostain.JPG)
Variants
- Epithelioid angiomyolipoma
- There is a special variant called an epithelioid angiomyolipoma, composed of epithelial looking cells, often with nuclear atypia
Cytologic
Cytologic features of angiomyolipoma include:[2]
Immunohistochemistry
Immunohistochemistry markers of angiomyolipoma include:
- Melanocytic markers positive[4]
- HMB-45 positive in all cases (15/15)[5]
- Melan A positive in ~87% of cases (13/15)
- Epithelial markers negative[4]
- EMA
- AE1/AE3
- SMA positive
- CD117 positive/negative
- Ki-67:[6]
- Epithelioid variant of angiomyolipoma positive
- Conventional angiomyolipoma negative
References
- ↑ Image courtesy of Dr Andrew Ryan. Radiopaedia (original file [1]).[http://radiopaedia.org/licence Creative Commons BY-SA-NC
- ↑ 2.0 2.1 Crapanzano, JP. (2005). "Fine-needle aspiration of renal angiomyolipoma: cytological findings and diagnostic pitfalls in a series of five cases". Diagn Cytopathol. 32 (1): 53–7. doi:10.1002/dc.20179. PMID 15584043. Unknown parameter
|month=ignored (help) - ↑ 3.0 3.1 3.2 3.3 http://librepathology.org/wiki/index.php/Angiomyolipoma
- ↑ 4.0 4.1 Template:Ref GUP
- ↑ Esheba, Gel S.; Esheba, Nel S. (2013). "Angiomyolipoma of the kidney: clinicopathological and immunohistochemical study". J Egypt Natl Canc Inst. 25 (3): 125–34. doi:10.1016/j.jnci.2013.05.002. PMID 23932749. Unknown parameter
|month=ignored (help) - ↑ Ooi, SM.; Vivian, JB.; Cohen, RJ. (2009). "The use of the Ki-67 marker in the pathological diagnosis of the epithelioid variant of renal angiomyolipoma". Int Urol Nephrol. 41 (3): 559–65. doi:10.1007/s11255-008-9473-1. PMID 18839327.