Wolfgang Hirsch, Carsten Bock (Halle)  

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Abdomen-Renals  

neoplastic  

The kidney changes are mostly likely found in the course of a tuberous cerebral sclerosis (Bourneville-Pringle's disease: Skin, cerebral and pulmonary changes). Sometimes also seen as a chance finding in kidney sonography (as first directing finding of a tuberous cerebral sclerosis). Very rare are kidney symptoms (i.e. Hematuria, palpable tumor).  

Composition of different parts of vessels, muscles and fat tissue. The presenting symptoms therefore vary in accordance to the composition of these tissues.

A single angiomyolipoma must not be indicative for Bourneville - Pringle's Disease.

See also:
Tuberous cerebral sclerosis;
Lymphangioleiomyomatosis.

 

<- view Ultrasound 1

Ultrasound 1: Multiple, echo rich small tumors. Also frequently associated with multiple cysts.



<- view Ultrasound 2

Ultrasound 2: Multiple, echo rich small tumors.



<- view MRI 1

MRT 1: (Coronal - FLASH 2D sequence with fat supression)



<- view MRI 2

MRT 2: (Transversal - FLASH 2D sequence with fat supression)

Hyperintense in T1 and T2, due to the high fat content. Often obvious enhancement (only in ares of high vasculature). Frequently well-seen in fat-reducing sequences.
CT: fat equivalent (i.e. negative HU-values in CT), occasionally however also denser representation (in higher vasculature or muscle).



 

Total constellation (Consens)  

If cystic parts predominate: polycystic kidney disease

Teratoma (otherwise difficult differentials, particularly if hemorrhaging changes the picture); angiomyelofibromas do not calcify, in contrast to teratomas.
Nephroblastomatosis  

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Angiomyolipoma, kidney, Bourneville-Pringle's disease, tuberous sclerosis of the brain, child, childhood, pediatric radiology  

Wolfgang Hirsch, Carsten Bock. Angiomyolipomas of the kidney in Bourneville-Pringle's disease. PedRad [serial online] vol 1, no. 11.
URL: www.PedRad.info/?search=20011129180447