Angiomyolipoma

Overview:

• Angiomyolipomas are type of benign neoplasm, most commonly found in tuberous sclerosis.
• They are composed of vascular, smooth muscle and fat element.
• Angiomyolipomas are the most common benign solid renal lesion and also the most common
fat-containing lesion of the kidneys. The majority of angiomyolipomas are sporadic and are
typically identified in adults, with a female predilection.
• The vast majority of angiomyolipoma are in the setting of tuberous sclerosis (80%)
although, they have also been described in the setting of Von Hippel-Lindau syndrome and
neurofibromatosis type 1.
 Angiomyolipoma
Pathophysiology:

• These are members of perivascular epitheloid cells tumor group and are composed of three
components:
i) Blood vessel
ii) Spindle cells
iii) Adipose tissue
Classic angiomyolipomas are benign but they do have a risk of rupture with bleeding or
secondary damage/destruction of surrounding structure as they grow.
 Angiomyolipoma
Clinical Presentation:

• Angiomyolipomas are an incidental finding and are asymptomatic.

• Symptomatic presentation most frequently occur with retroperitoneal hemorrhage where
patient may undergo shock.
• The other symptoms are:
• i) Palpable mass
• Ii) Flank pain
• Iii) Urinary tract infection
• Iv) Hematuria
• V) Renal Failure
• Vi) Hypertension
 Angiomyolipoma
Radiological Presentation:

• To find angiomyolipoma on all the modalities macroscopic fat is identified.

• In the setting of hemorrhage the lesion contains little fat and it is difficult to distinguish
angiomyolipoma from renal cell carcinoma.

• Ultrasound
• Lesion appear hyperechoic.
• In the setting of tuberous sclerosis entire kidney is affected they appear echogenic along with
loss of normal corticomedullary differentiation.
 Angiomyolipoma
Radiological Presentation:

CT:
• Most of the lesion involve cortex and demonstrate macroscopic fat (<20 HU).
• 5% of angiomyolipoma are fat-poor especially in the setting of tuberous sclerosis these do
not demonstrate macroscopic fat on CT.

MRI:
• It is an excellent tool for evaluating fat containing lesions.
• Angiomyolipomas demonstrate high signal intensity.
Angiomyolipoma: Ultrasound

US image shows fat-rich angiomyolipoma (arrows) is

more hyperechoic than right renal sinus (asterisk).
Angiomyolipoma: Ultrasound

Fat-poor angiomyolipoma (arrows) with

heterogeneous echotexture. Lesion echogenicity
(asterisk) is less than that of right renal sinus.
 Angiomyolipoma: CT

Unenhanced CT image (left) shows large solid

left renal mass (arrows).

Contrast-enhanced CT image (right) depicts

tortuous well-enhancing vessels within lesion
(arrows).
 Angiomyolipoma: MRI

Axial T2-weighted MR image (left) shows fat-rich

AML (arrows) is hyperintense owing to large
amount of fat (black asterisk).
Axial fat-suppressed T2-weighted MR image
(right) clearly depicts suppression of fat (white
asterisk), which is hyperintense on T2-weighted
MR images.
For reference:
1. https://www.ajronline.org/doi/full/10.2214/AJR.17.17973
2. https://radiopaedia.org/articles/renal-angiomyolipoma?lang=us