Changes to This Summary (03/29/2012)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

General Information ¹

Added text ² to state that patients who develop Wilms tumor in their second decade of life have a poorer survival than younger patients with Wilms tumor (cited Popov et al. as reference 8).

Added text ³ to state that germline inactivating mutations in DIS3L2 on chromosome 2q37 are associated with Perlman syndrome (cited Astuti et al. as reference 18).

Added text ⁴ to state that approximately 10% of patients with Beckwith-Wiedemann syndrome will develop a malignancy, with either Wilms tumor or hepatoblastoma being the most common, although adrenal tumors can also occur (cited Tan et al. as reference 28).

Added text ⁵ to state that features associated with germline WT1 mutations that increase the risk for developing renal failure are stromal predominant histology, bilaterality, intralobular nephrogenic rests, and Wilms tumor diagnosed before age 2 years (cited Lange et al. as reference 34).

Cellular Classification ⁶

Added text ⁷ to state that tumors occurring in older patients have a higher incidence of anaplastic histology (cited Popov et al. as reference 2).

Added text ⁸ to state that recommendations for surveillance in patients with germline SMARCB1 mutations have been developed based on the epidemiology and clinical course of rhabdoid tumors. From birth to age 1 year, it is suggested that patients have thorough physical and neurologic examinations, as well as head ultrasounds monthly. It is suggested that patients undergo abdominal ultrasounds with focus on the kidneys every 2 to 3 months. From age 1 year to approximately age 4 years, it is suggested that brain and spine magnetic resonance imaging and abdominal ultrasound be performed every 6 months.

Treatment of Renal Cell Carcinoma ⁹

Added text ¹⁰ to state that a case report of an adolescent with a TFE-3 renal cell carcinoma suggests responsiveness to multiple tyrosine kinase inhibitors (cited De Pasquale et al. as reference 5).

Treatment of Recurrent Wilms Tumor and Other Childhood Kidney Tumors ¹¹

Added text ¹² about the COG-ADVL1121 clinical trial as a treatment option under clinical evaluation.