Angiomyolipoma
Angiomyolipoma
Updated: 10/06/2020
© Jun Wang, MD, PhD
General features
- Usually benign neoplasm
- Admixture of blood vessels with hyalinization, smooth muscle and adipose tissue
- Seen in other organs liver, lungs, retroperitoneal soft tissue
- May be associated with tuberous sclerosis
- Death may be caused by local invasion, especially to blood vessels
Clinical presentations
- Most asymptomatic with normal renal function
- Flank pain
- Gross hematuria
- Retroperitoneal hemorrhage may occur
- Renal failure if bilateral
Pathological findings
- Three components: vessel, smooth muscle, adipose tissue
- Torturous thick-walled vessels
- Myoid spindle cells
- Mature adipose tissue
- May be pleomorphic, suggestive of aggressive behavior
Genetic abnormalities
- TSC2 / PKD1
Marker
- Positive for HMB45
Diagnosis
- Image studies
- Biopsy
Management
- Life threatening hemorrhage: Selective renal artery embolization
- Nephrectomy, nephron-sparing surgery and radiofrequency ablation
- Screening for tuberous sclerosis: high risk if multiple, bilateral, or larger (ie, ≥4 cm) tumors; family history, review of system and physical exam
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