Nephroblastomas, aka, Wilms tumors

The most common renal cancer in children ages 2-4; they are the most common pediatric cancer overall. A large, palpable flank mass, hematuria, and hypertension. Nephroblastomas are associated with several childhood syndromes caused by mutations on chromosome 11, including:

• WAGR complex (Wilms tumor, Aniridia, Genitourinary malformation, Range of developmental delays; WT1 deletion)

• Denys-Drash syndrome (Wilms tumor, diffuse mesangial sclerosis, male pseudohermaphroditism.; WTI mutation)

• Beckwith-Wiedemann syndrome (Wilms tumor, macroglossia, organomegaly, hemihyperplasia, omphalocele; alterations in genes that control body growth on chromosome 11).

Prognosis depends in part on the histopathology:

• "Favorable" histology is characterized by embryonic glomerular structures with blastema, epithelial tubules, and mesenchyme (stroma).

• "Unfavorable" histology is characterized by more anaplasia; we'll see hyperchromatic, enlarged pleomorphic nuclei with abnormal mitotic figures.

With favorable histology, prognosis is good; treatment includes surgery and radiation therapy.