Christopher P. Coppola, Alfred P. Kennedy, Jr. and Ronald J. Scorpio (eds.)Pediatric Surgery2014Diagnosis and Treatment10.1007/978-3-319-04340-1_69
© Springer International Publishing Switzerland 2014
Wilms’ Tumor
(1)
Department of Haematology/Oncology, Janet Weis Children’s Hospital, 100 N. Academy Av. MC 13-20, Danville, PA 17822, USA
1.
Pathophysiology.
(a)
Renal masses in children:
(i)
5 % of cancer diagnosis below 20 years.
(ii)
550 children/year diagnosed in USA.
(iii)
Wilms’ tumor most common (95 %). Most commonly seen below 5-years-old.
(iv)
Rhabdoid tumors 1 %, primarily in infants and have bone metastasis.
(v)
Clear cell sarcoma of kidney (2 %), usually below 5-years-old and have brain metastasis.
(vi)
Renal cell carcinoma: Most common in adults, only 2 % occur in patients below 20-years-old.
(b)
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Conditions associated with Wilms’ tumor:
(i)
GU anomalies (6 %): Denys-Drash syndrome, intersex disorders, mesangial sclerosis, and Wilms tumor predisposition.
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