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{{Infobox_Disease |
'''For patient information, click [[{{PAGENAME}} (patient information)|here]].'''
  Name          = {{PAGENAME}} |
  Image          = bilateralrenalAML.jpg |
  Caption        = |
  DiseasesDB    = 29496 |
  ICD10          = {{ICD10|D|30|0|d|10}} |
  ICD9          = {{ICD9|223.0}} |
  ICDO          = M8860/0 |
  OMIM          = |
  MedlinePlus    = |
  eMedicineSubj  = |
  eMedicineTopic = |
  MeshID        = D018207 |
}}
{{Angiomyolipoma}}
{{Angiomyolipoma}}
{{CMG}}''' Associate Editor-In-Chief:''' {{CZ}}
{{CMG}}''' Associate Editor-In-Chief:''' {{CZ}}, {{Faizan}}, {{Rekha}}<br>
{{SK}} Renal hamartoma, AML, renal angiomyolipoma, renal AML, renal angiomyolipomata, angiomyolipoma of the kidney


==Overview==
==[[Angiomyolipoma overview|Overview]]==


'''Angiomyolipoma''' is a benign [[kidney|renal]] neoplasm previously considered to be a [[hamartoma]] or choristoma, but now known to be [[neoplastic]].<ref>Eble JN. Angiomyolipoma of the kidney. Semin Diagn Pathol 1988;15:21-40</ref> It is composed of variable amounts of [[fat]], [[Blood vessel|vascular]], and [[smooth muscle]] elements. The fat density of the tumour on [[CT]] has been regarded to be [[pathognomonic]], although there are now case reports of renal cell carcinoma types also possessing macroscopic fat.
==[[Angiomyolipoma historical perspective|Historical Perspective]]==


The lesion is well demarcated and contains mature elements. It occurs in more than 50% of individuals with [[tuberous sclerosis]], often bilaterally. Angiomyolipomata also occur in 40% of women who have a rare, cystic lung disease called lymphangioleiomyomatosis, or LAM.<ref name="pmid16915145">{{cite journal |author=McCormack FX |title=Lymphangioleiomyomatosis |journal=MedGenMed |volume=8 |issue=1 |pages=15 |year=2006 |pmid=16915145 |pmc=1682009 |doi= |url=http://www.medscape.com/viewarticle/518317}}</ref>
==[[Angiomyolipoma classification|Classification]]==


==Incidence==
==[[Angiomyolipoma pathophysiology|Pathophysiology]]==


The incidence is about 0.3-3%. It occurs in more than 50% of individuals with [[tuberous sclerosis]], often bilaterally. It is twice as common in females as in males.
==[[Angiomyolipoma causes|Causes]]==


==Types==
==[[Angiomyolipoma differential diagnosis|Differentiating Angiomyolipoma from Other Diseases]]==


* Isolated Angiomyolipoma (80%)
==[[Angiomyolipoma epidemiology and demographics|Epidemiology and Demographics]]==
** Angiomyolipoma is about 4 times more common in women than in men.
** Interestingly, 80% of the cases involve the right kidney.
* Angiomyolipoma associated with [[tuberous sclerosis]] (20%).
** Angiomyolipoma occurs in 80% patients with [[tuberous sclerosis]].
** Angiomyolipoma also occurs young women with lymphangiomyomatosis without other stigmata of [[tuberous sclerosis]]. Angiomyolipoma and lymphangiomyomatosis are sometimes considered ''the forme fruste'' of [[tuberous sclerosis]].


==Diagnosis==
==[[Angiomyolipoma risk factors|Risk Factors]]==


Demonstration of fat in renal tumor is virtually diagnostic of angiomyolipoma. Most small lesions are asymptomatic and incidental findings on images. The fat density of the tumour on [[CT]] is [[pathognomonic]].
==[[Angiomyolipoma screening|Screening]]==


===Diagnostic Findings===
==[[Angiomyolipoma natural history, complications and prognosis|Natural History, Complications and Prognosis]]==


[http://www.radswiki.net Images courtesy of RadsWiki]
==Diagnosis==
 
[[Image:Angiomyolipoma 101.jpg|thumb|left|350px|MRI: Angiomyolipoma]]
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[[Image:Angiomyolipoma 102.jpg|thumb|left|350px|MRI: Angiomyolipoma]]
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[[Image:Angiomyolipoma 103.jpg|thumb|left|350px|US: Angiomyolipoma]]
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[[Image:Angiomyolipoma 104.jpg|thumb|left|350px|CT: Angiomyolipoma]]
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[[Image:Angiomyolipoma_001.jpg|thumb|left|350px|CT: Large right angiomyolipoma]]
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[[Image:Angiomyolipoma 002.jpg|thumb|left|350px|CT: Large right angiomyolipoma]]
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'''US and CT images demonstrate a large liver angiomyolipoma'''
<gallery>
Image:
 
Liver angiomyolipoma 001.jpg
 
Image:
 
Liver angiomyolipoma 002.jpg
 
Image:
 
Liver angiomyolipoma 003.jpg
 
Image:
 
Liver angiomyolipoma 004.jpg
 
</gallery>
 
===Histopathological Findings===
 
[[Image:Renal angiomyolipoma (1).jpg|thumb|left|350px|1. Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. H & E stain.]]
<br clear="left"/>


[[Image:Renal angiomyolipoma (2).jpg|thumb|left|350px|2. Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. The same case as demonstrated in "Image 1". H & E stain.]]
[[Angiomyolipoma diagnostic study of choice|Diagnostic Study of Choice]] | [[Angiomyolipoma history and symptoms|History and Symptoms ]] | [[ Angiomyolipoma physical examination|Physical Examination]] | [[Angiomyolipoma laboratory findings|Laboratory Findings]] | [[Angiomyolipoma electrocardiogram|Electrocardiogram]] | [Angiomyolipoma x-ray|X-ray]] | [[Angiomyolipoma CT|CT]] | [[Angiomyolipoma MRI|MRI]] | [[Angiomyolipoma ultrasound|Ultrasound]] | [[Angiomyolipoma other imaging findings|Other Imaging Findings]] | [[Angiomyolipoma other diagnostic studies|Other Diagnostic Studies]]
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[[Image:Renal angiomyolipoma (3).jpg|thumb|left|350px|3. Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. The same case as demonstrated in "Image 1". H & E stain.]]
==Treatment==
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[[Angiomyolipoma medical therapy|Medical Therapy]] | [[Angiomyolipoma surgery |Surgery]] | [[Angiomyolipoma primary prevention|Primary Prevention]] | [[Angiomyolipoma secondary prevention|Secondary Prevention]] | [[Angiomyolipoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] |  [[Angiomyolipoma future or investigational therapies|Future or Investigational Therapies]]


[[Image:Renal angiomyolipoma (4) HMB-45 immunostain.JPG|thumb|left|350px|4. Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. The same case as demonstrated in "Image 1". HMB-45 immunostain.]]
==Case Studies==
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==Complications==
[[Angiomyolipoma case study one|Case #1]]
 
Complications include [[hematuria]], [[flank pain]], and [[shock]] as a result of spontaneous [[hemorrhage]].


==References==
==References==
{{reflist|2}}
{{reflist|2}}


==See also==
==Related Chapters==
*[[PEComa]]
*[[PEComa]]
==External links==
* {{Chorus|00488}}
* [http://www.thelamfoundation.org/ The LAM Foundation (US)]




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Latest revision as of 10:45, 11 September 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2], Faizan Sheraz, M.D. [3], Rekha, M.D.
Synonyms and keywords: Renal hamartoma, AML, renal angiomyolipoma, renal AML, renal angiomyolipomata, angiomyolipoma of the kidney

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Angiomyolipoma from Other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | [Angiomyolipoma x-ray|X-ray]] | CT | MRI | Ultrasound | Other Imaging Findings | Other Diagnostic Studies

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