ANGIOMYOLIPOMA WITH INTRATUMORAL PSUEDOANEURYSM AND HAEMORRHAGE

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 A 33 year old female patient presented with right flank pain for 2 days with no history of hematuria. CECT abdomen was done 

              3D volume reconstructed images shows the  pseudo aneurysm in the right renal area

Contrast enhanced axial CT abdomen image shows a heterogenous lesion in the mid pole of right kidney with fat densities within  an intensely enhancing rounded intratumoral pseudo aneurysm. Hyperdense areas also noted within the lesion suggestive of  haemorrhage within the tumour ...above mentioned features are suggestive of right renal angiomyolipoma with intratumoral pseudo aneurysm and haemorrhage  within 



Coronal image shows the same findings in mid pole of right kidney.

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                                                              Sagittal images of the same   



USG shows a heterogenous right renal inter polar mass with hyperechoic components and a round hypoechoic intratumoral area.
 

i
  The round intratumoral area shows bidirectional colour flow (red and blue) signal on colour Doppler study,  suggestive of pseudo-aneurysm

Renal angiomyolipoma 

It’s is the most common solid tumor of the kidney. There is excess growth of smooth muscles and thick walled blood vessels and mature fat. They are classified under perivascular epitheloid cell proliferation(PEComas). Most commonly associated syndrome is tuberous sclerosis. Multiple bilateral angiomyolipoma s suggest possibility of tuberous sclerosis.

Ultrasonography

Highly echogenic mass (more echogenic than central fat). It may appear as echogenic fat within a tumour or isolated foci of echogenicity. If fat content is less of mixed with blood echogenicity of tumour is reduced and can mimic other tumours of kidney. Posterior shadowing may be seen in angiomyolipoma which can be a differentiating feature with that of renal cell carcinoma.

CT scan

Most accurate in AML. Macroscopic fat can be seen. AML are usually well marginated but do not have a true capsule. They are usually at a margin of the kidney. Intratumoral aneurysm may be present.

MRI

Can also demonstrate fat component. On MRI AMLs have high T1 signal and on fat saturated images a drop in signal is noted. On opposed phase images characteristic Indian ink artefact is seen. Lipid poor AML is usually hypointense on T2 images but RCC s will be T2 hyper intense  which will help in differentiating both.

Angiography may demonstrate multiple aneurysms and onion layer appearance.

Management                                                          

Annual follow up is recommended for asymptomatic lesions less than 4 cm. For larger lesions due to risk of hemorrhage semiannual evaluation is suggested.

Small solitary lesions less than 2 cm do not require follow up 


Larger AMLs, or those that have been symptomatic, can be electively embolized and/or resected with a partial nephrectomy.

Lesions that present with retroperitoneal hemorrhage often require emergency embolization as a life-saving measure.


Differential Diagnosis 

  1. RCC -  Macroscopic fat if seen is associated with calcification. Microscopic fat will not show Indian ink artefact in out phase.
  2. Retroperitoneal liposarcoma - hypovascular 
  3. Adrenal myelolipoma



 

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