- What is the diagnosis?
This is a Wilms tumor of the kidney. Approximately 10% of all Wilms tumors carry mutations in the WT1 gene which has been identified as a tumor suppressor gene. The WAGR (Wilms' tumor with aniridia, genitourinary malformations, and retardation) syndrome is associated with a deletion within the short arm of one copy of chromosome 11 where the WT1 gene is located.
- What is the peak age incidence of this tumor?
Wilms tumor is seen most often between ages 2 and 4. Wilms tumor is the most common intra-abdominal solid tumor of childhood, affecting 1 in 10,000 children worldwide.
- What is the survival of patients with this tumor today?
Wilms tumor therapy is a major success story in medicine. With careful workup and treatment, over 90% of patients with Wilms tumor can be cured using contemporary multimodality therapy (surgery, radiation, and chemotherapy).
- How do you deal with poor outcomes?
Even though the survival rate with Wilms tumor and other pediatric neoplasms is now very good, there are treatment failures. The natural question is: is there hospice for NICU or PICU age patients? The answer is that it is unusual. The pediatric community recognizes this need for the most part, but there are powerful psychological forces at work: parents just can't stand the thought of losing a child, and tend to want to 'try everything.' Even professionals like pediatricians often don't think it is natural for children to die, like it is for the elderly, and so resist talking about end-of-life care. And many good and well meaning hospice volunteers are prepared to take care of adults or the elderly, but would find the tragedy of the death of a child hard to bear. Having a designated place for such an event, such as an extra large private room where the family can all be there with the baby or child, is useful in these situations. And pain medications should always be readily available.
