Angiomyolipoma: Difference between revisions

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{{CMG}}''' Associate Editor-In-Chief:''' {{CZ}}
{{CMG}}''' Associate Editor-In-Chief:''' {{CZ}}


==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 natural history|Natural History, Complications and Prognosis]]==


===Diagnostic Findings===
==Diagnosis==
 
[http://www.radswiki.net Images courtesy of RadsWiki]
 
[[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'''
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Image:
 
Liver angiomyolipoma 001.jpg
 
Image:
 
Liver angiomyolipoma 002.jpg
 
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Liver angiomyolipoma 003.jpg
 
Image:
 
Liver angiomyolipoma 004.jpg
 
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===Histopathological Findings===
 
[[Image:Renal angiomyolipoma (1).jpg|thumb|left|350px|1. Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. H & E stain.]]
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[[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.]]
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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.]]
[[Angiomyolipoma history and symptoms|History and Symptoms ]] | [[ Angiomyolipoma physical examination|Physical Examination]] | [[Angiomyolipoma laboratory findings|Laboratory Findings]] | [[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 (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.]]
==Treatment==
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[[Angiomyolipoma medical therapy|Medical Therapy]] | [[Angiomyolipoma surgery |Surgery]] | [[Angiomyolipoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] |  [[Angiomyolipoma future or investigational therapies|Future or Investigational Therapies]]


==Complications==
==Case Studies==


Complications include [[hematuria]], [[flank pain]], and [[shock]] as a result of spontaneous [[hemorrhage]].
[[Angiomyolipoma case study one|Case #1]]


==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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[[Category:Types of cancer]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Oncology]]
[[Category:Disease]]


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Revision as of 13:57, 11 September 2012

Angiomyolipoma
ICD-10 D30.0
ICD-9 223.0
ICD-O: M8860/0
DiseasesDB 29496
MeSH D018207

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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]

Overview

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Pathophysiology

Causes

Differentiating Angiomyolipoma from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | CT | MRI | Ultrasound | Other Imaging Findings | Other Diagnostic Studies

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