Seeing the musical, Les Miserables, was a great experience for me. In one of my favorite moments, a little girl appears on the stage alone and sings a hauntingly beautiful solo, "There is a castle on a cloud..."

 

I like to go there in my sleep.
Aren't any floors for me to sweep,
Not in my castle on a cloud...

There is a lady all in white,
Holds me and sings a lullaby.
She's nice to see
And she's soft to touch.
She says, "Cosette, I love you very much."

I know a place where no one's lost.
I know a place where no one cries.
Crying at all is not allowed,
Not in my castle on a cloud.

The real little girl who played the role of young Cosette was living a dream come true. She was a star!

I met her shortly after seeing the show. While she was appearing in the musical, she developed a bothersome cough that was making it difficult to sing. When she came in to have it checked out, the physical exam revealed a large mass in her belly, obscuring her kidney. Her chest X-ray showed that her lungs were filled with cancer and causing her to cough. The little girl who sang poignantly of a place where no one cries had her fairy tale life interrupted when she learned that she had Wilms' tumor.

Wilms' tumor, sometimes called Nephroblastoma, is by far the most common of the pediatric kidney cancers. It occurs in about approximately 8 children per million children younger than 15 years (Nelson Textbook of Pediatrics, 17th Ed). The most common age at diagnosis is 2 to 5 years, just as children are bursting with the exuberant energy of discovering their own preferences and desires. Ninety percent of cases are diagnosed before children have completed first grade.

While Wilms’ tumor is the most common of the pediatric kidney cancers, it is not the only one. Clear cell sarcoma of the kidney is the second most common kidney cancer in children, followed by several much less common cancers such as Rhabdoid Tumor of the kidney and Congenital Mesoblastic Nephroma.

Let’s talk a bit more about what we know about Wilms’ tumor. Most of us have at least two genes, both located on the 11th chromosome, that prevent our kidney cells from growing out of control. These genes are called WT1 and WT2 (for Wilms’ tumor suppressing genes). When our cells reproduce, the DNA molecules, or blueprints, are copied for the new cells. Sometimes, the copy is not perfect. Sometimes, the DNA is damaged by exposure to radiation or to chemical carcinogens. Often this damage is repaired, but not always.

Kids who develop Wilms' tumor have both of these genes knocked out. In some families, one of these genes is missing from the outset. All it takes, then, is one insult to the other gene to produce the tumor. In familial cases, the tumor is more likely to occur in both kidneys. It is also more likely to be associated with other kidney/urinary problems and with two other unusual physical findings: Hemihypertrophy (one side of the body is larger than the other) and aniridia (the absence of the iris -- the colored part of the eye). [Note -- this is a bit of an oversimplification.

Show full page