Difference between revisions of "Angiomyolipoma"

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Angiomyolipoma (view source)

Revision as of 05:46, 25 July 2014

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#redirect [[Kidney_tumours#Angiomyolipoma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Angiomyolipoma.PEComa.JPG
| Width      =
| Caption    = Angiomyolipoma. [[H&E stain]].
| Synonyms  =
| Micro      = smooth muscle, adipose tissue (not always present), abundant blood vessels.
| Subtypes  = conventional, epithelioid angiomyolipoma
| LMDDx      = [[clear cell renal cell carcinoma]] (esp. for epithelioid variant)
| Stains    =
| IHC        = HMB-45 +ve, Melan A +ve, SMA +ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[kidney]] - see ''[[kidney tumours]]''
| Assdx      =
| Syndromes  = [[tuberous sclerosis]]
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = uncommon
| Bloodwork  =
| Rads      = classically has regions consistent with fat
| Endoscopy  =
| Prognosis  = benign, epithelioid variant may be aggressive
| Other      =
| ClinDDx    = other [[kidney tumours]]
| Tx        =
}}
'''Angiomyolipoma''', abbreviated '''AML''', is a benign mesenchymal tumour of the [[kidney tumours|kidney]] that is associated with [[tuberous sclerosis]] and belongs to the [[PEComas|PEComa group of tumours]].
 
==General==
*Benign mesenchymal tumour.
*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>
*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.
*In the [[PEComa]] group of tumours.
 
===Epidemiology===
*May be associated with [[tuberous sclerosis]] -- 70% have an AML.
**When compared to sporadic cases:
***More often bilateral.
***Usually bigger.
*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>
 
==Microscopic==
Features:
*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'''.
*Abundant blood vessels.
 
===Epithelioid angiomyolipoma===
Features:
*Carcinoma-like morphology.
*+/-Spindle cells.
*"High grade" nuclei.
**Pleomorphic nuclei.
 
DDx:
*[[Clear cell renal cell carcinoma]] eosinophilic variant - esp. if epithelioid.
 
Images:
*[http://bjr.birjournals.org/content/82/984/e249/F3.expansion.html Epithelioid AML (birjournals.org)].
*[http://radiographics.rsna.org/content/30/6/1525/F2.expansion.html Epithelioid AML (rsna.org)].
*[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>
 
===Cytologic===
Features<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>
*Nuclei - round/ovoid.
*Chromatin - bland.
 
==IHC==
*Melanocytic markers +ve.<ref name=Ref_GUP324>{{Ref GUP|324}}</ref>
**HMB-45 +ve in all cases (15/15).<ref name=pmid23932749>{{Cite journal  | last1 = Esheba | first1 = Gel S. | last2 = Esheba | first2 = Nel S. | title = Angiomyolipoma of the kidney: clinicopathological and immunohistochemical study. | journal = J Egypt Natl Canc Inst | volume = 25 | issue = 3 | pages = 125-34 | month = Sep | year = 2013 | doi = 10.1016/j.jnci.2013.05.002 | PMID = 23932749 }}
</ref>
**Melan A +ve in ~87% of cases (13/15).
*Epithelial markers -ve.<ref name=Ref_GUP324>{{Ref GUP|324}}</ref>
*SMA +ve.
*CD117 +ve/-ve.
 
*Ki-67:<ref name=pmid18839327>{{Cite journal  | last1 = Ooi | first1 = SM. | last2 = Vivian | first2 = JB. | last3 = Cohen | first3 = RJ. | title = The use of the Ki-67 marker in the pathological diagnosis of the epithelioid variant of renal angiomyolipoma. | journal = Int Urol Nephrol | volume = 41 | issue = 3 | pages = 559-65 | month =  | year = 2009 | doi = 10.1007/s11255-008-9473-1 | PMID = 18839327 }}</ref>
**Epithelioid variant of AML +ve.
**Conventional AML -ve.
 
==See also==
*[[Kidney tumours]].
*[[PEComas]].
 
==References==
{{Reflist|2}}
 
[[Category:Diagnosis]]
[[Category:Kidney tumours]]
Michael
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