Difference between revisions of "Angiomyolipoma"

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Angiomyolipoma (view source)

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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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*Presentations: flank pain, hematuria, incidentaloma.<ref name=pmid18805573>{{Cite journal  | last1 = Seyam | first1 = RM. | last2 = Bissada | first2 = NK. | last3 = Kattan | first3 = SA. | last4 = Mokhtar | first4 = AA. | last5 = Aslam | first5 = M. | last6 = Fahmy | first6 = WE. | last7 = Mourad | first7 = WA. | last8 = Binmahfouz | first8 = AA. | last9 = Alzahrani | first9 = HM. | title = Changing trends in presentation, diagnosis and management of renal angiomyolipoma: comparison of sporadic and tuberous sclerosis complex-associated forms. | journal = Urology | volume = 72 | issue = 5 | pages = 1077-82 | month = Nov | year = 2008 | doi = 10.1016/j.urology.2008.07.049 | PMID = 18805573 }}</ref>
*Presentations: flank pain, hematuria, incidentaloma.<ref name=pmid18805573>{{Cite journal  | last1 = Seyam | first1 = RM. | last2 = Bissada | first2 = NK. | last3 = Kattan | first3 = SA. | last4 = Mokhtar | first4 = AA. | last5 = Aslam | first5 = M. | last6 = Fahmy | first6 = WE. | last7 = Mourad | first7 = WA. | last8 = Binmahfouz | first8 = AA. | last9 = Alzahrani | first9 = HM. | title = Changing trends in presentation, diagnosis and management of renal angiomyolipoma: comparison of sporadic and tuberous sclerosis complex-associated forms. | journal = Urology | volume = 72 | issue = 5 | pages = 1077-82 | month = Nov | year = 2008 | doi = 10.1016/j.urology.2008.07.049 | PMID = 18805573 }}</ref>
**Tumours >4 cm considered a risk for bleeding.<ref name=pmid21571778>{{Cite journal  | last1 = Abrams | first1 = J. | last2 = Yee | first2 = DC. | last3 = Clark | first3 = TW. | title = Transradial embolization of a bleeding renal angiomyolipoma. | journal = Vasc Endovascular Surg | volume = 45 | issue = 5 | pages = 470-3 | month = Jul | year = 2011 | doi = 10.1177/1538574411408352 | PMID = 21571778 }}</ref>
**Tumours >4 cm considered a risk for bleeding.<ref name=pmid21571778>{{Cite journal  | last1 = Abrams | first1 = J. | last2 = Yee | first2 = DC. | last3 = Clark | first3 = TW. | title = Transradial embolization of a bleeding renal angiomyolipoma. | journal = Vasc Endovascular Surg | volume = 45 | issue = 5 | pages = 470-3 | month = Jul | year = 2011 | doi = 10.1177/1538574411408352 | PMID = 21571778 }}</ref>
**May bleed spontaneously ([[Wunderlich's syndrome]]).<ref name=pmid19104113>{{Cite journal  | last1 = Moratalla | first1 = MB. | title = Wunderlich's syndrome due to spontaneous rupture of large bilateral angiomyolipomas. | journal = Emerg Med J | volume = 26 | issue = 1 | pages = 72 | month = Jan | year = 2009 | doi = 10.1136/emj.2008.062091 | PMID = 19104113 }}</ref>
*AMLs occur may be elsewhere in the body, e.g. liver,<ref name=pmid15498214>{{Cite journal  | last1 = Zhang | first1 = SH. | last2 = Cong | first2 = WM. | last3 = Xian | first3 = ZH. | last4 = Wu | first4 = WQ. | last5 = Dong | first5 = H. | last6 = Wu | first6 = MC. | title = [Morphologic variants and immunohistochemical features of hepatic angiomyolipoma.] | journal = Zhonghua Bing Li Xue Za Zhi | volume = 33 | issue = 5 | pages = 437-40 | month = Oct | year = 2004 | doi =  | PMID = 15498214 }}
*AMLs occur may be elsewhere in the body, e.g. liver,<ref name=pmid15498214>{{Cite journal  | last1 = Zhang | first1 = SH. | last2 = Cong | first2 = WM. | last3 = Xian | first3 = ZH. | last4 = Wu | first4 = WQ. | last5 = Dong | first5 = H. | last6 = Wu | first6 = MC. | title = [Morphologic variants and immunohistochemical features of hepatic angiomyolipoma.] | journal = Zhonghua Bing Li Xue Za Zhi | volume = 33 | issue = 5 | pages = 437-40 | month = Oct | year = 2004 | doi =  | PMID = 15498214 }}
</ref> but are most common in the periadrenal and perirenal fat.
</ref> but are most common in the periadrenal and perirenal fat.
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**This is not confirmed by all studies.<ref name=pmid18852677>{{Cite journal  | last1 = Aydin | first1 = H. | last2 = Magi-Galluzzi | first2 = C. | last3 = Lane | first3 = BR. | last4 = Sercia | first4 = L. | last5 = Lopez | first5 = JI. | last6 = Rini | first6 = BI. | last7 = Zhou | first7 = M. | title = Renal angiomyolipoma: clinicopathologic study of 194 cases with emphasis on the epithelioid histology and tuberous sclerosis association. | journal = Am J Surg Pathol | volume = 33 | issue = 2 | pages = 289-97 | month = Feb | year = 2009 | doi = 10.1097/PAS.0b013e31817ed7a6 | PMID = 18852677 }}</ref>
**This is not confirmed by all studies.<ref name=pmid18852677>{{Cite journal  | last1 = Aydin | first1 = H. | last2 = Magi-Galluzzi | first2 = C. | last3 = Lane | first3 = BR. | last4 = Sercia | first4 = L. | last5 = Lopez | first5 = JI. | last6 = Rini | first6 = BI. | last7 = Zhou | first7 = M. | title = Renal angiomyolipoma: clinicopathologic study of 194 cases with emphasis on the epithelioid histology and tuberous sclerosis association. | journal = Am J Surg Pathol | volume = 33 | issue = 2 | pages = 289-97 | month = Feb | year = 2009 | doi = 10.1097/PAS.0b013e31817ed7a6 | PMID = 18852677 }}</ref>
*More common in women than men - both in sporadic AMLs and those associated with tuberous sclerosis.<ref name=pmid17003820>{{cite journal |author=Rakowski SK, Winterkorn EB, Paul E, Steele DJ, Halpern EF, Thiele EA |title=Renal manifestations of tuberous sclerosis complex: Incidence, prognosis, and predictive factors |journal=Kidney Int. |volume=70 |issue=10 |pages=1777–82 |year=2006 |month=November |pmid=17003820 |doi=10.1038/sj.ki.5001853 |url=}}</ref>
*More common in women than men - both in sporadic AMLs and those associated with tuberous sclerosis.<ref name=pmid17003820>{{cite journal |author=Rakowski SK, Winterkorn EB, Paul E, Steele DJ, Halpern EF, Thiele EA |title=Renal manifestations of tuberous sclerosis complex: Incidence, prognosis, and predictive factors |journal=Kidney Int. |volume=70 |issue=10 |pages=1777–82 |year=2006 |month=November |pmid=17003820 |doi=10.1038/sj.ki.5001853 |url=}}</ref>
==Gross==
*Well circumscribed - uniform yellow.
Note:
*Small resected AML are often fat poor.
*May resemble a renal cell carcinoma on gross.
Radiology:
*<=10 HU in an intermediate size tumour.<ref name=pmid21555349>{{Cite journal  | last1 = Davenport | first1 = MS. | last2 = Neville | first2 = AM. | last3 = Ellis | first3 = JH. | last4 = Cohan | first4 = RH. | last5 = Chaudhry | first5 = HS. | last6 = Leder | first6 = RA. | title = Diagnosis of renal angiomyolipoma with hounsfield unit thresholds: effect of size of region of interest and nephrographic phase imaging. | journal = Radiology | volume = 260 | issue = 1 | pages = 158-65 | month = Jul | year = 2011 | doi = 10.1148/radiol.11102476 | PMID = 21555349 }}</ref>
Image:
*[http://webpathology.com/image.asp?n=6&Case=72 AML (webpathology.com)].


==Microscopic==
==Microscopic==
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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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===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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====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 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.


==See also==
==See also==
Michael
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