Wilms' tumor pathophysiology
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Pathophysiology
Pathologically, a triphasic nephroblastoma comprises three elements:
Wilms' tumor is a malignant tumor containing metanephric blastema, stromal and epithelial derivatives. Characteristic is the presence of abortive tubules and glomeruli surrounded by a spindled cell stroma. The stroma may include striated muscle, cartilage, bone, fat tissue, fibrous tissue. The tumor is compressing the normal kidney parenchyma.
Wilms’ tumor is thought to arise from pre-existing lesions known as nephrogenic rests. In unilateral tumors, nephrogenic rests might be a prognostic factor for recurrence in the opposite kidney. Nephrogenic rests may be hyperplastic (masses) or schlerotic (fibrous) and may have immature tubular forms.
They commonly have a capsule and are vascular. They rarely cross the midline. Metastases are most often to the lungs.
The mesenchymal component may include cells showing rhabdomyoid differentiation. The rhabdomyoid component may itself show features of malignancy (rhabdomyosarcomatous Wilms).
Wilms tumor may be separated into 2 prognostic groups based on pathologic characteristics:
- Favorable - Contains well developed components mentioned above
- Anaplastic - Contains diffuse anaplasia (poorly developed cells)