Angiomyolipoma
| Angiomyolipoma | |
 | |
|---|---|
| ICD-10 | D30.0 |
| ICD-9 | 223.0 |
| ICD-O: | M8860/0 |
| DiseasesDB | 29496 |
| MeSH | D018207 |
|
WikiDoc Resources for Angiomyolipoma |
|
Articles |
|---|
|
Most recent articles on Angiomyolipoma Most cited articles on Angiomyolipoma |
|
Media |
|
Powerpoint slides on Angiomyolipoma |
|
Evidence Based Medicine |
|
Clinical Trials |
|
Ongoing Trials on Angiomyolipoma at Clinical Trials.gov Trial results on Angiomyolipoma Clinical Trials on Angiomyolipoma at Google
|
|
Guidelines / Policies / Govt |
|
US National Guidelines Clearinghouse on Angiomyolipoma NICE Guidance on Angiomyolipoma
|
|
Books |
|
News |
|
Commentary |
|
Definitions |
|
Patient Resources / Community |
|
Patient resources on Angiomyolipoma Discussion groups on Angiomyolipoma Patient Handouts on Angiomyolipoma Directions to Hospitals Treating Angiomyolipoma Risk calculators and risk factors for Angiomyolipoma
|
|
Healthcare Provider Resources |
|
Causes & Risk Factors for Angiomyolipoma |
|
Continuing Medical Education (CME) |
|
International |
|
|
|
Business |
|
Experimental / Informatics |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Please Take Over This Page and Apply to be Editor-In-Chief for this topic: There can be one or more than one Editor-In-Chief. You may also apply to be an Associate Editor-In-Chief of one of the subtopics below. Please mail us [2] to indicate your interest in serving either as an Editor-In-Chief of the entire topic or as an Associate Editor-In-Chief for a subtopic. Please be sure to attach your CV and or biographical sketch.
Overview
Angiomyolipoma is a benign renal neoplasm previously considered to be a hamartoma or choristoma, but now known to be neoplastic.[1] It is composed of variable amounts of fat, 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.
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.[2]
Incidence
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.
Types
- Isolated Angiomyolipoma (80%)
- 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
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.
Diagnostic Findings
Additional images
-
Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. H & E stain. -
Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. The same case as demonstrated in a file "Renal_angiomyolipoma_(1).jpg". H & E stain. -
Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. The same case as demonstrated in a file "Renal_angiomyolipoma_(1).jpg". H & E stain. -
Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. The same case as demonstrated in a file "Renal_angiomyolipoma_(1).jpg". HMB-45 immunostain.
.jpg)
.jpg)
.jpg)
_HMB-45_immunostain.JPG)
Complications
Complications include hematuria, flank pain, and shock as a result of spontaneous hemorrhage.