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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
| Subtypes  = conventional, epithelioid angiomyolipoma
| Subtypes  = conventional, epithelioid angiomyolipoma
| LMDDx      = [[clear cell renal cell carcinoma]] (esp. for epithelioid variant)
| LMDDx      = [[clear cell renal cell carcinoma]] (esp. for epithelioid variant), [[Xp11.2 translocation carcinoma]] (esp. for epithelioid variant), well-differentiated retroperitoneal [[sarcoma]] (esp. [[leiomyosarcoma]] & [[liposarcoma]]), [[renal cell carcinoma with sarcomatoid differentiation]], [[renal leiomyoma]]
| Stains    =
| Stains    =
| IHC        = HMB-45 +ve, Melan A +ve, SMA +ve
| IHC        = HMB-45 +ve, Melan A +ve, SMA +ve, PAX8 -ve, CK (pooled) -ve
| EM        =
| EM        =
| Molecular  =
| Molecular  =
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| Gross      =
| Gross      =
| Grossing  =
| Grossing  =
| Site      = [[kidney]] - see ''[[kidney tumours]]''
| Site      = [[kidney]] (see ''[[kidney tumours]]''), other sites
| Assdx      =
| Assdx      =
| Syndromes  = [[tuberous sclerosis]]
| Syndromes  = [[tuberous sclerosis]]
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| Other      =
| Other      =
| ClinDDx    = other [[kidney tumours]]
| ClinDDx    = other [[kidney tumours]]
| Tx        =
| Tx        = surgery - esp. if large or imaging characteristics ambiguous
}}
}}
'''Angiomyolipoma''', abbreviated '''AML''', is a benign mesenchymal tumour that is associated with [[tuberous sclerosis]] and belongs to the [[PEComas|PEComa group of tumours]]. It is typically found in the [[kidney]].
'''Angiomyolipoma''', abbreviated '''AML''', is a benign mesenchymal tumour that is associated with [[tuberous sclerosis]] and belongs to the [[PEComas|PEComa group of tumours]].  
 
It is typically found in the [[kidney]]; however, occasionally it is seen extrarenal.<ref name=pmid24555133>{{Cite journal  | last1 = Minja | first1 = EJ. | last2 = Pellerin | first2 = M. | last3 = Saviano | first3 = N. | last4 = Chamberlain | first4 = RS. | title = Retroperitoneal extrarenal angiomyolipomas: an evidence-based approach to a rare clinical entity. | journal = Case Rep Nephrol | volume = 2012 | issue =  | pages = 374107 | month =  | year = 2012 | doi = 10.1155/2012/374107 | PMID = 24555133 }}</ref>


==General==
==General==
Line 36: Line 38:
*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 kidney.
</ref> but are most common in the periadrenal and perirenal fat.
*In the [[PEComa]] group of tumours.
*In the [[PEComa]] group of tumours.


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***More often bilateral.
***More often bilateral.
***Usually bigger.
***Usually bigger.
**Reported to often have estrogen and progesterone receptors - in the context of [[lymphangioleiomyomatosis]].<ref name=pmid10631194>{{Cite journal  | last1 = Logginidou | first1 = H. | last2 = Ao | first2 = X. | last3 = Russo | first3 = I. | last4 = Henske | first4 = EP. | title = Frequent estrogen and progesterone receptor immunoreactivity in renal angiomyolipomas from women with pulmonary lymphangioleiomyomatosis. | journal = Chest | volume = 117 | issue = 1 | pages = 25-30 | month = Jan | year = 2000 | doi =  | PMID = 10631194 }}</ref>
*There is a suggestion that an ''epithelioid'' variant is more worrisome.<ref name=pmid12352384>{{Cite journal  | last1 = Nelson | first1 = CP. | last2 = Sanda | first2 = MG. | title = Contemporary diagnosis and management of renal angiomyolipoma. | journal = J Urol | volume = 168 | issue = 4 Pt 1 | pages = 1315-25 | month = Oct | year = 2002 | doi = 10.1097/01.ju.0000028200.86216.b2 | PMID = 12352384 }}</ref>
*There is a suggestion that an ''epithelioid'' variant is more worrisome.<ref name=pmid12352384>{{Cite journal  | last1 = Nelson | first1 = CP. | last2 = Sanda | first2 = MG. | title = Contemporary diagnosis and management of renal angiomyolipoma. | journal = J Urol | volume = 168 | issue = 4 Pt 1 | pages = 1315-25 | month = Oct | year = 2002 | doi = 10.1097/01.ju.0000028200.86216.b2 | PMID = 12352384 }}</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>
**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>
==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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*Smooth muscle.
*Smooth muscle.
*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 vessels.
*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:
*Retroperitoneal [[sarcoma]].
**[[Liposarcoma]].
**[[Leiomyosarcoma]].
*[[Renal cell carcinoma with sarcomatoid differentiation]].
*[[Renal leiomyoma]] - very rare - desmin +ve, HMB-45 -ve.<ref name=pmid25517956>{{Cite journal  | last1 = Patil | first1 = PA. | last2 = McKenney | first2 = JK. | last3 = Trpkov | first3 = K. | last4 = Hes | first4 = O. | last5 = Montironi | first5 = R. | last6 = Scarpelli | first6 = M. | last7 = Nesi | first7 = G. | last8 = Aron | first8 = M. | last9 = Sangoi | first9 = AR. | title = Renal Leiomyoma: A Contemporary Multi-institution Study of an Infrequent and Frequently Misclassified Neoplasm. | journal = Am J Surg Pathol | volume =  | issue =  | pages =  | month = Dec | year = 2014 | doi = 10.1097/PAS.0000000000000354 | PMID = 25517956 }}</ref>


===Images===
===Images===
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Image: Renal_angiomyolipoma_(1).jpg | AML (WC/KGH).
Image: Renal_angiomyolipoma_(1).jpg | AML (WC/KGH).
Image: Renal_angiomyolipoma_(2).jpg | AML (WC/KGH).
Image: Renal_angiomyolipoma_(2).jpg | AML (WC/KGH).
Image: Adrenal Angiomyolipoma MP CTR.jpg|Angiomyolipoma (SKB)
Image: Kidney Angiomyolipoma Hemorrhage HP CTR.jpg|Renal angiomyolipoma with hemorrhage (SKB)
Image: Kidney Angiomyolipoma Hemorrhage HP2 CTR.jpg|Renal angiomyolipoma with hemorrhage (SKB)
Image: Kidney Angiomyolipoma HP CTR (2).jpg|Renal angiomyolipoma (SKB)
Image: Kidney Angiomyolipoma 4 LP CTR.jpg|Renal angiomyolipoma - low power (SKB)
Image: Kidney Angiomyolipoma 4 MP2 CTR.jpg|Renal angiomyolipoma- medium power (SKB)
Image: Kidney Angiomyolipoma Hemorrhage LP CTR.jpg|Renal angiomyolipoma - low power (SKB)
Image: Kidney Angiomyolipoma 4 MP CTR.jpg|Renal angiomyolipoma - medium power (SKB)
Image: Kidney Angiomyolipoma Hemorrhage MP CTR.jpg|Renal angiomyolipoma - medium power (SKB)
Image: Kidney Angiomyolipoma 1 PA.jpg|Renal angiomyolipoma - Tumor cells stream off a blood vessel (SKB) 
Image: Kidney Angiomyolipoma MP CTR.jpg|Renal Angiomyolipoma - medium power (SKB)
Image: Kidney Angiomyolipoma HP CTR.jpg|Renal angiomyolipoma - high power (SKB)
Image: Kidney Angiomyolipoma MP2 CTR.jpg|Renal Angiomyolipoma - medium power (SKB)
Image: Kidney Angiomyolipoma MP CTR (2).jpg|Renal Angiomyolipoma - medium power (SKB)
Image: Kidney Angiomyolipoma MP CTR (3).jpg|Renal Angiomyolipoma - medium power (SKB)
Image: Kidney Angiomyolipoma SolidType HP PA.jpg|Renal Angiomyolipoma - solid type - high power (SKB)
Image: Kidney Angiomyolipoma SolidType HP2 PA.jpg|Renal angiomyolipoma - solid type (SKB)
Image: Kidney Angiomyolipoma SolidVariant MP3 CTR.jpg|Renal angiomyolipoma - solid type (SKB)
Image: Kidney Angiomyolipoma SolidVariant MP CTR.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)
</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>


Line 69: Line 127:


DDx:
DDx:
*[[Clear cell renal cell carcinoma]] eosinophilic variant - esp. if epithelioid.
*[[Clear cell renal cell 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>


Images:
====Images====
*[http://bjr.birjournals.org/content/82/984/e249/F3.expansion.html Epithelioid AML (birjournals.org)].
<gallery>
*[http://radiographics.rsna.org/content/30/6/1525/F2.expansion.html Epithelioid AML (rsna.org)].
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/modpathol201372f2.html#figure-title Epithelioid AML - spectrum of patterns (nature.com)].<ref name=pmid23599151/>
*[http://www.nature.com/modpathol/journal/v26/n10/fig_tab/modpathol201372f1.html#figure-title Epithelioid AML - variants (nature.com)].<ref name=pmid23599151/>
*[http://images.rsna.org/index.html?doi=10.1148/rg.306105517&fig=fig07a Epithelioid AML (rsna.org)].<ref name=pmid21071373>{{Cite journal  | last1 = Katabathina | first1 = VS. | last2 = Vikram | first2 = R. | last3 = Nagar | first3 = AM. | last4 = Tamboli | first4 = P. | last5 = Menias | first5 = CO. | last6 = Prasad | first6 = SR. | title = Mesenchymal neoplasms of the kidney in adults: imaging spectrum with radiologic-pathologic correlation. | journal = Radiographics | volume = 30 | issue = 6 | pages = 1525-40 | month = Oct | year = 2010 | doi = 10.1148/rg.306105517 | PMID = 21071373 }}</ref>
*[http://www.archivesofpathology.org/action/showFullPopup?id=i1543-2165-128-10-1176-f01&doi=10.1043%2F1543-2165%282004%29128%3C1176%3APQCAYW%3E2.0.CO%3B2 Atypical epithelioid AML (archivesofpathology.org)].<ref>{{Cite journal  | last1 = Aljerian | first1 = K. | last2 = Evans | first2 = AJ. | title = Pathologic quiz case: a 44-year-old woman with an incidental asymptomatic renal mass. Atypical epithelioid angiomyolipoma. | journal = Arch Pathol Lab Med | volume = 128 | issue = 10 | pages = 1176-8 | month = Oct | year = 2004 | doi = 10.1043/1543-2165(2004)1281176:PQCAYW2.0.CO;2 | PMID = 15387699 }}</ref>
*[http://www.archivesofpathology.org/action/showFullPopup?id=i1543-2165-128-10-1176-f01&doi=10.1043%2F1543-2165%282004%29128%3C1176%3APQCAYW%3E2.0.CO%3B2 Atypical epithelioid AML (archivesofpathology.org)].<ref>{{Cite journal  | last1 = Aljerian | first1 = K. | last2 = Evans | first2 = AJ. | title = Pathologic quiz case: a 44-year-old woman with an incidental asymptomatic renal mass. Atypical epithelioid angiomyolipoma. | journal = Arch Pathol Lab Med | volume = 128 | issue = 10 | pages = 1176-8 | month = Oct | year = 2004 | doi = 10.1043/1543-2165(2004)1281176:PQCAYW2.0.CO;2 | PMID = 15387699 }}</ref>


Line 86: Line 158:
</ref>
</ref>
**Melan A +ve in ~87% of cases (13/15).
**Melan A +ve in ~87% of cases (13/15).
*Epithelial markers -ve.<ref name=Ref_GUP324>{{Ref GUP|324}}</ref>
*Epithelial markers -ve<ref name=Ref_GUP324>{{Ref GUP|324}}</ref>, e.g. EMA and AE1/AE3.
*SMA +ve.
*SMA +ve.
*CD117 +ve/-ve.
*CD117 +ve/-ve.
Line 93: Line 165:
**Epithelioid variant of AML +ve.
**Epithelioid variant of AML +ve.
**Conventional AML -ve.
**Conventional AML -ve.
A panel:
*CK (pooled), Desmin, Melan A, HMB-45.
===Epithelioid AML versus Xp11.2 translocation carcinoma===
[[ISUP]] recommends:<ref name=pmid25025364 >{{cite journal |author=Amin MB, Epstein JI, Ulbright TM, ''et al.'' |title=Best practices recommendations in the application of immunohistochemistry in urologic pathology: report from the international society of urological pathology consensus conference |journal=Am. J. Surg. Pathol. |volume=38 |issue=8 |pages=1017–22 |year=2014 |month=August |pmid=25025364 |doi=10.1097/PAS.0000000000000254 |url=}}</ref>
*PAX8 -ve.
**Positive in Xp11.2 translocation carcinoma.
*CAIX -ve.
*AE1/AE3 -ve.
*EMA -ve.
==Sign out==
<pre>
RIGHT KIDNEY, PARTIAL NEPHRECTOMY:
- ANGIOMYOLIPOMA.
</pre>
====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.
===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>
MASS OF LEFT KIDNEY, CORE BIOPSY:
- COMPATIBLE WITH ANGIOMYOLIPOMA.
COMMENT:
The tumour stains as follows:
POSITIVE: Melan A, HMB-45, desmin.
NEGATIVE: CK (pooled).
</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.


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