Angiomyolipoma (AML): Benign Kidney Tumor – Symptoms, Diagnosis, Treatment
Angiomyolipoma (AML) is a benign mesenchymal tumor composed of blood vessels, smooth muscle, and adipose tissue.
Epidemiology
- Most common in kidney (renal AML); also liver, spleen, colon, lung.
- Strong association with tuberous sclerosis complex (TSC) (bilateral, multiple, younger onset).
- Sporadic: usually solitary, women > men, 40s–60s.
Clinical Presentation
- Asymptomatic (majority): Incidental on imaging for other reasons.
- Symptomatic (large >4 cm): Flank/abdominal pain, palpable mass, hematuria.
- Life-threatening: Spontaneous retroperitoneal hemorrhage (Wunderlich syndrome) – risk ↑ with size >4 cm, pregnancy, TSC.
Diagnosis
- Ultrasound: Hyperechoic (fat), may shadow.
- CT (gold standard): Fat density (<-20 HU) diagnostic. Enhancing soft tissue components = vessels/muscle.
- MRI: Fat signal on T1; chemical shift artifact.
- Biopsy: Rarely needed if imaging classic; risk of bleeding.
Management
- Surveillance: <4 cm, asymptomatic – periodic imaging.
- Embolization (first-line for >4 cm / symptomatic): Selective angiographic embolization – preserves renal parenchyma.
- Surgery: Partial nephrectomy (preferred), radical if necessary. For failed embolization, suspicion of malignancy, non-embolizable anatomy.
- mTOR inhibitors (sirolimus): For TSC-associated AMLs (shrinks tumors).
Prognosis
Excellent for sporadic AML. TSC patients require lifelong multidisciplinary care. Malignant transformation (epithelioid AML) is rare.
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