Difference between revisions of "Angiomyolipoma"

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(redirect to renal tumour for now)
 
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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]]

Revision as of 05:46, 25 July 2014

Angiomyolipoma
Diagnosis in short

Angiomyolipoma. H&E stain.
LM smooth muscle, adipose tissue (not always present), abundant blood vessels.
Subtypes conventional, epithelioid angiomyolipoma
LM DDx clear cell renal cell carcinoma (esp. for epithelioid variant)
IHC HMB-45 +ve, Melan A +ve, SMA +ve
Site kidney - see kidney tumours
Syndromes tuberous sclerosis
Prevalence uncommon
Radiology classically has regions consistent with fat
Prognosis benign, epithelioid variant may be aggressive
Clin. DDx other kidney tumours

Angiomyolipoma, abbreviated AML, is a benign mesenchymal tumour of the kidney that is associated with tuberous sclerosis and belongs to the PEComa group of tumours.

General

  • Benign mesenchymal tumour.
  • Presentations: flank pain, hematuria, incidentaloma.[1]
    • Tumours >4 cm considered a risk for bleeding.[2]
  • AMLs occur may be elsewhere in the body, e.g. liver,[3] 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.[4]
    • This is not confirmed by all studies.[5]

Microscopic

Features:

  • Smooth muscle.
  • Adipose tissue - not always present[6] - key feature.
  • Abundant blood vessels.

Epithelioid angiomyolipoma

Features:

  • Carcinoma-like morphology.
  • +/-Spindle cells.
  • "High grade" nuclei.
    • Pleomorphic nuclei.

DDx:

Images:

Cytologic

Features[6]

  • Nuclei - round/ovoid.
  • Chromatin - bland.

IHC

  • Melanocytic markers +ve.[8]
    • HMB-45 +ve in all cases (15/15).[9]
    • Melan A +ve in ~87% of cases (13/15).
  • Epithelial markers -ve.[8]
  • SMA +ve.
  • CD117 +ve/-ve.
  • Ki-67:[10]
    • Epithelioid variant of AML +ve.
    • Conventional AML -ve.

See also

References

  1. ↑ Seyam, RM.; Bissada, NK.; Kattan, SA.; Mokhtar, AA.; Aslam, M.; Fahmy, WE.; Mourad, WA.; Binmahfouz, AA. et al. (Nov 2008). "Changing trends in presentation, diagnosis and management of renal angiomyolipoma: comparison of sporadic and tuberous sclerosis complex-associated forms.". Urology 72 (5): 1077-82. doi:10.1016/j.urology.2008.07.049. PMID 18805573.
  2. ↑ Abrams, J.; Yee, DC.; Clark, TW. (Jul 2011). "Transradial embolization of a bleeding renal angiomyolipoma.". Vasc Endovascular Surg 45 (5): 470-3. doi:10.1177/1538574411408352. PMID 21571778.
  3. ↑ Zhang, SH.; Cong, WM.; Xian, ZH.; Wu, WQ.; Dong, H.; Wu, MC. (Oct 2004). "[Morphologic variants and immunohistochemical features of hepatic angiomyolipoma.]". Zhonghua Bing Li Xue Za Zhi 33 (5): 437-40. PMID 15498214.
  4. ↑ Nelson, CP.; Sanda, MG. (Oct 2002). "Contemporary diagnosis and management of renal angiomyolipoma.". J Urol 168 (4 Pt 1): 1315-25. doi:10.1097/01.ju.0000028200.86216.b2. PMID 12352384.
  5. ↑ Aydin, H.; Magi-Galluzzi, C.; Lane, BR.; Sercia, L.; Lopez, JI.; Rini, BI.; Zhou, M. (Feb 2009). "Renal angiomyolipoma: clinicopathologic study of 194 cases with emphasis on the epithelioid histology and tuberous sclerosis association.". Am J Surg Pathol 33 (2): 289-97. doi:10.1097/PAS.0b013e31817ed7a6. PMID 18852677.
  6. ↑ 6.0 6.1 Crapanzano, JP. (Jan 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.
  7. ↑ Aljerian, K.; Evans, AJ. (Oct 2004). "Pathologic quiz case: a 44-year-old woman with an incidental asymptomatic renal mass. Atypical epithelioid angiomyolipoma.". Arch Pathol Lab Med 128 (10): 1176-8. doi:10.1043/1543-2165(2004)1281176:PQCAYW2.0.CO;2. PMID 15387699.
  8. ↑ 8.0 8.1 Zhou, Ming; Magi-Galluzzi, Cristina (2006). Genitourinary Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 324. ISBN 978-0443066771.
  9. ↑ Esheba, Gel S.; Esheba, Nel S. (Sep 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.
  10. ↑ 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.
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