Tuberous sclerosis with renal AML

Case contributed by Dr Jake Ball

Presentation

60 year old presents with reduced GCS with abdominal pain.

Patient Data

Age: 60 years
Gender: Male

CT Brain

CT

Multiple cortical tubers and subependymal nodules. 

Extensive white matter hypodensity.

Left eye prosthesis.

CT Angiogram - Abdominal

CT

The background left and right kidney are enlarged with heterogenous fat containing lesion throughout. 

Large hematoma in the right kidney with no evidence of active bleed. Abrupt cut-off of a right renal artery is seen in keeping with prior embolization history. No pseudoaneurysms are noted. 

No retroperitoneal cysts.
Remainder of solid viscera are normal with no hepatic AMLs.

Sclerotic bone lesions throughout. 

Case Discussion

This case demonstrates multiple facets of tuberous sclerosis with bilateral AML and sclerotic bony lesions. Interestingly left eye prosthesis in keeping with previous retinal phakoma. 

There has been interim removal of the right kidney due to ongoing hemorrhage, a complication of enlarging renal AML.

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