Difference between revisions of "Angiomyolipoma"

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{{ Infobox diagnosis
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Name      = {{PAGENAME}}
| Image      = Renal_angiomyolipoma_(1).jpg  
| Image      = Angiomyolipoma -- low mag.jpg  
| Width      =
| Width      =
| Caption    = Angiomyolipoma. [[H&E stain]].
| Caption    = [[Micrograph]] of an angiomyolipoma - showing the three components (blood vessels (''angio-'') - left top, muscle (''myo-'') - elsewhere, fat (''lipoma'') - right bottom). [[H&E stain]].
| Synonyms  =
| Synonyms  =
| Micro      = smooth muscle, adipose tissue (not always present), abundant blood vessels
| Micro      = smooth muscle, adipose tissue (not always present), abundant blood vessels
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Note:
Note:
*Small resected AML are often fat poor.
*Small resected AML are often fat poor.
*May resemble a renal cell carcinoma on gross.


Radiology:
Radiology:
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*Adipose tissue - not always present<ref name=pmid15584043>{{Cite journal  | last1 = Crapanzano | first1 = JP. | title = Fine-needle aspiration of renal angiomyolipoma: cytological findings and diagnostic pitfalls in a series of five cases. | journal = Diagn Cytopathol | volume = 32 | issue = 1 | pages = 53-7 | month = Jan | year = 2005 | doi = 10.1002/dc.20179 | PMID = 15584043 }}</ref> - '''key feature'''.
*Adipose tissue - not always present<ref name=pmid15584043>{{Cite journal  | last1 = Crapanzano | first1 = JP. | title = Fine-needle aspiration of renal angiomyolipoma: cytological findings and diagnostic pitfalls in a series of five cases. | journal = Diagn Cytopathol | volume = 32 | issue = 1 | pages = 53-7 | month = Jan | year = 2005 | doi = 10.1002/dc.20179 | PMID = 15584043 }}</ref> - '''key feature'''.
*Abundant [[blood vessel]]s.
*Abundant [[blood vessel]]s.
Notes:
*May have melanin pigment - occasionally extensive.<ref name=pmid26118214>{{Cite journal  | last1 = Reddy | first1 = R. | last2 = Lewin | first2 = JR. | last3 = Shenoy | first3 = V. | title = Pigmented Epithelioid Angiomyolipoma of the Kidney. | journal = J Miss State Med Assoc | volume = 56 | issue = 4 | pages = 92-4 | month = Apr | year = 2015 | doi =  | PMID = 26118214 }}</ref><ref name=pmid23136579>{{Cite journal  | last1 = Chang | first1 = H. | last2 = Jung | first2 = W. | last3 = Kang | first3 = Y. | last4 = Jung | first4 = WY. | title = Pigmented perivascular epithelioid cell tumor (PEComa) of the kidney: a case report and review of the literature. | journal = Korean J Pathol | volume = 46 | issue = 5 | pages = 499-502 | month = Oct | year = 2012 | doi = 10.4132/KoreanJPathol.2012.46.5.499 | PMID = 23136579 }}</ref>
*May involve lymph nodes - case reports.<ref name=pmid2294869>{{cite journal |authors=Ro JY, Ayala AG, el-Naggar A, Grignon DJ, Hogan SF, Howard DR |title=Angiomyolipoma of kidney with lymph node involvement. DNA flow cytometric analysis |journal=Arch Pathol Lab Med |volume=114 |issue=1 |pages=65–7 |date=January 1990 |pmid=2294869 |doi= |url=}}</ref><ref name=pmid12696187>{{cite journal |vauthor=Shiga Y, Tsutsumi M, Suzuki K, Ishikawa S, Shimogama T |title=Angiomyolipoma with regional lymph node involvement: a case report and literature review |journal=Hinyokika Kiyo |volume=49 |issue=2 |pages=81–6 |date=February 2003 |pmid=12696187 |doi= |url=}}</ref>


DDx:
DDx:
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Image: Kidney Angiomyolipoma SolidType HP3 PA.jpg|Renal angiomyolipoma - solid type (SKB)  
Image: Kidney Angiomyolipoma SolidType HP3 PA.jpg|Renal angiomyolipoma - solid type (SKB)  
Image: Kidney Angiomyolipoma SolidVariant MP2 CTR.jpg|Renal angiomyolipoma - solid type (SKB)  
Image: Kidney Angiomyolipoma SolidVariant MP2 CTR.jpg|Renal angiomyolipoma - solid type (SKB)  
</gallery>
====Case====
<gallery>
Image: Angiomyolipoma -- low mag.jpg | AML - low mag.
Image: Angiomyolipoma -- intermed mag.jpg | AML - intermed. mag.
Image: Angiomyolipoma - alt -- intermed mag.jpg | AML - intermed. mag.
Image: Angiomyolipoma -- high mag.jpg | AML - intermed. mag.
Image: Angiomyolipoma - alt -- high mag.jpg | AML - intermed. mag.
</gallery>
</gallery>


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DDx:
DDx:
*[[Clear cell renal cell carcinoma]] eosinophilic variant - esp. if epithelioid.
*[[Clear cell renal cell carcinoma]].
*[[Xp11.2 translocation carcinoma]].
*[[Xp11.2 translocation carcinoma]].
*Renal cell carcinoma with angioleiomyoma-like stroma.<ref name=pmid25189644>{{Cite journal  | last1 = Williamson | first1 = SR. | last2 = Cheng | first2 = L. | last3 = Eble | first3 = JN. | last4 = True | first4 = LD. | last5 = Gupta | first5 = NS. | last6 = Wang | first6 = M. | last7 = Zhang | first7 = S. | last8 = Grignon | first8 = DJ. | title = Renal cell carcinoma with angioleiomyoma-like stroma: clinicopathological, immunohistochemical, and molecular features supporting classification as a distinct entity. | journal = Mod Pathol | volume =  | issue =  | pages =  | month = Sep | year = 2014 | doi = 10.1038/modpathol.2014.105 | PMID = 25189644 }}</ref>
*Renal cell carcinoma with angioleiomyoma-like stroma.<ref name=pmid25189644>{{Cite journal  | last1 = Williamson | first1 = SR. | last2 = Cheng | first2 = L. | last3 = Eble | first3 = JN. | last4 = True | first4 = LD. | last5 = Gupta | first5 = NS. | last6 = Wang | first6 = M. | last7 = Zhang | first7 = S. | last8 = Grignon | first8 = DJ. | title = Renal cell carcinoma with angioleiomyoma-like stroma: clinicopathological, immunohistochemical, and molecular features supporting classification as a distinct entity. | journal = Mod Pathol | volume =  | issue =  | pages =  | month = Sep | year = 2014 | doi = 10.1038/modpathol.2014.105 | PMID = 25189644 }}</ref>


====Images====
====Images====
www:
<gallery>
Image: Epithelioid angiomyolipoma -- very low mag.jpg | EMAL - very low mag.
Image: Epithelioid angiomyolipoma -- low mag.jpg | EMAL - low mag.
Image: Epithelioid angiomyolipoma -- intermed mag.jpg | EMAL - intermed. mag.
Image: Epithelioid angiomyolipoma -- high mag.jpg | EMAL - high mag.
Image: Epithelioid angiomyolipoma -- very high mag.jpg | EMAL - very high mag.
Image: Epithelioid angiomyolipoma - alt -- low mag.jpg | EMAL - low mag.
Image: Epithelioid angiomyolipoma - alt -- intermed mag.jpg | EMAL - intermed. mag.
</gallery>
=====www=====
*[http://www.nature.com/modpathol/journal/v26/n10/fig_tab/modpathol201372f4.html#figure-title Epithelioid angiomyolipoma (nature.com)].<ref name=pmid23599151>{{Cite journal  | last1 = He | first1 = W. | last2 = Cheville | first2 = JC. | last3 = Sadow | first3 = PM. | last4 = Gopalan | first4 = A. | last5 = Fine | first5 = SW. | last6 = Al-Ahmadie | first6 = HA. | last7 = Chen | first7 = YB. | last8 = Oliva | first8 = E. | last9 = Russo | first9 = P. | title = Epithelioid angiomyolipoma of the kidney: pathological features and clinical outcome in a series of consecutively resected tumors. | journal = Mod Pathol | volume = 26 | issue = 10 | pages = 1355-64 | month = Oct | year = 2013 | doi = 10.1038/modpathol.2013.72 | PMID = 23599151 }}</ref>
*[http://www.nature.com/modpathol/journal/v26/n10/fig_tab/modpathol201372f4.html#figure-title Epithelioid angiomyolipoma (nature.com)].<ref name=pmid23599151>{{Cite journal  | last1 = He | first1 = W. | last2 = Cheville | first2 = JC. | last3 = Sadow | first3 = PM. | last4 = Gopalan | first4 = A. | last5 = Fine | first5 = SW. | last6 = Al-Ahmadie | first6 = HA. | last7 = Chen | first7 = YB. | last8 = Oliva | first8 = E. | last9 = Russo | first9 = P. | title = Epithelioid angiomyolipoma of the kidney: pathological features and clinical outcome in a series of consecutively resected tumors. | journal = Mod Pathol | volume = 26 | issue = 10 | pages = 1355-64 | month = Oct | year = 2013 | doi = 10.1038/modpathol.2013.72 | PMID = 23599151 }}</ref>
*[http://www.nature.com/modpathol/journal/v26/n10/fig_tab/modpathol201372f2.html#figure-title Epithelioid AML - spectrum of patterns (nature.com)].<ref name=pmid23599151/>
*[http://www.nature.com/modpathol/journal/v26/n10/fig_tab/modpathol201372f2.html#figure-title Epithelioid AML - spectrum of patterns (nature.com)].<ref name=pmid23599151/>
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====Micro====
====Micro====
The section show a circumscribed tumour composed of muscle-like tissue and adipose tissue with prominent muscular blood vessels.  Rare nuclear enlargement is seen. No signficant nuclear atypia is appreciated. Mitotic activity is not apparent.
The section show a circumscribed tumour composed of muscle-like tissue and adipose tissue with prominent muscular blood vessels.  Rare nuclear enlargement is seen. No significant nuclear atypia is appreciated. Mitotic activity is not apparent.


===Biopsy===
===Biopsy===
<pre>
Left Kidney, Mass Lesion, Core Biopsy:
    - Angiomyolipoma, see comment.
Comment:
The tumour stains as follow:
POSITIVE: melan A (focal, rare).
NEGATIVE: PAX8, AE1/AE3, HMB-45, desmin, PR, ER.
</pre>
====Alternate====
<pre>
<pre>
MASS OF LEFT KIDNEY, CORE BIOPSY:
MASS OF LEFT KIDNEY, CORE BIOPSY:
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NEGATIVE: CK (pooled).
NEGATIVE: CK (pooled).
</pre>
</pre>
=====Micro=====
The sections show a tumour composed of spindle cells in a fascicular arrangement with scant adipose tissue and prominent muscular blood vessels.  Rare nuclear enlargement is seen. No significant nuclear atypia is appreciated. Mitotic activity is not apparent. No renal parenchyma is identified.
===Epithelioid===
Comment:
Epithelioid change in angiomyolipoma can be associated with aggressive behaviour; follow-up is recommended.


====Micro====
====Micro====
The sections show a tumour composed of spindle cells in a fasicular arrangement with scant adipose tissue and prominent muscular blood vessels. Rare nuclear enlargement is seen. No signficant nuclear atypia is appreciated. Mitotic activity is not apparent. No renal parenchyma is identified.
The sections show a tumour composed of spindle cells in a fascicular arrangement with abundant adipose tissue and prominent muscular blood vessels. A focal epithelioid subcomponent is present and has moderate atypia. Mitotic activity is not readily apparent. The epithelioid component is completely removed in the plane-of-section.


==See also==
==See also==
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