Nephroblastomatose

Toddler with
a left abdominal mass. The diagnosis was Wilms tumor of the left kidney and nephrogenic rests from nephroblastomatosis in the right kidney.The diagnosis was Wilms tumor of the left kidney and nephrogenic rests from nephroblastomatosis in the right kidney.
pediatricimaging.org • CC-by-nc-sa 4.0
Radiopaedia • CC-by-nc-sa 3.0 • de

Nephroblastomatosis refers to diffuse or multifocal involvement of the kidneys with nephrogenic rests (persistent metanephric blastema).

Epidemiology

Nephrogenic rests are found incidentally in 1% of infants.

Pathology

Nephrogenic rests are foci of metanephric blastema that persist beyond 36 weeks of gestation and have the potential for malignant transformation into Wilms tumor.

It is currently believed that nephrogenic rests give rise to approximately 30-40% of Wilms tumors. Nephrogenic rests are found in up to 99% of bilateral Wilms tumors.

There are two pathologic subtypes of nephrogenic rest, perilobar rest (90%) and intralobar rest (10%), the latter being more associated with Wilms tumor.

Associations

Radiographic features

Ultrasound

Ultrasound may demonstrate hypoechoic nodules but is less sensitive than MR imaging and CT. Diffuse nephroblastomatosis is usually seen as reniform enlargement with a thick peripheral rind of tissue that may show striated enhancement. On ultrasound, the enlarged kidney may have diffusely decreased echogenicity.

CT

At CT, macroscopic nephrogenic rests appear as low-attenuation peripheral nodules with poor enhancement relative to that of adjacent normal renal parenchyma.

MRI

Typical signal characteristics include:

  • T1: the nodules demonstrate low-signal-intensity foci
  • T2: the nodules demonstrate low-signal-intensity foci
  • T1 C+ (Gd): hypointense mass enhances less than renal tissue

Treatment and prognosis

Treatment for nephrogenic rests is controversial. Some investigators recommend chemotherapy, whereas others maintain that close serial radiologic evaluation of enlarging masses is sufficient.

Differential diagnosis

  • renal lymphoma can mimic the appearance of nephroblastomatosis but is unusual in infants and young children.

See also

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu Nephroblastomatose:
Lymphom der
Niere
Wikipedia • CC BY-SA 3.0
Nephroblastom
 
pediatricimaging.org • CC-by-nc-sa 4.0
Hemihyperplasie
 
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