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
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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