Hereditary Kidney Wilms Tumor

**Semantic type:** Neoplastic Process

**Definition:** Familial embryonal neoplasm derived from nephrogenic blastemal cells. Several lines of differentiation, including blastemal, stromal and epithelial, are usually expressed. Comprises approximately 1% of Wilms tumors. (AFIP fascicle version 2.0)

**Synonyms:** - Familial Kidney Wilms Tumor - Hereditary Kidney Adenosarcoma - Hereditary Kidney Nephroblastoma - Hereditary Renal Adenosarcoma - Hereditary Wilms Tumor