The last word on Wilms tumor
Every day, three or four Federal Express packages arrive at the office of
pathologist Elizabeth J. Perlman, MD. By
day's end, Perlman has reviewed the dozens of slides in each package and
prepared a report confirming or correcting the diagnosis of kidney cancer.
Perlman, professor of pathology at Northwestern University's Feinberg School of
Medicine and head of the Department of Pathology and Laboratory Medicine at
Children's Memorial Hospital, arrived from Johns Hopkins University in February
2002. With her, she brought the National Wilms Tumor Study Group Pathology
Center, which reviews 90 percent of the 450 cases of Wilms tumor diagnosed
annually, as well as other pediatric kidney tumors.
The most common kidney tumor in children
Wilms tumor is the most common kidney tumor in children, accounting for 90
percent of kidney cancers, and 6 percent of all pediatric cancers. As reviewing
pathologist for the center, Perlman examines samples from tumors removed during
surgery, and establishes not only the diagnosis of cancer type, but the staging
of the tumor. "A lot of the tumors are evaluated by pathologists at local
hospitals, who may see one Wilms tumor in a lifetime," says Perlman. "So, the
rate of incorrect diagnoses, including staging diagnoses, is almost 25 percent."
Accuracy in diagnosis and staging is vital to placing each child on the correct
protocol, a sequence of treatments that may include chemotherapy, radiation, or
both. Treatment is successful in 85 to 90 percent of cases.
Precise staging and carefully measured outcomes are
key
The group oversees clinical trials on therapies and develops treatment
protocols for Wilms tumor and other kidney cancers. Treatment of pediatric
cancer requires particularly precise staging and careful measurement of
treatment outcomes to limit the long-term side effects of radiation and
chemotherapy, which can cause hearing loss, infertility, and even secondary
cancers in adult life. The National Wilms Tumor Study Group has developed
effective protocols while reducing treatment. The latest protocol, in use soon
for very early-stage tumors of infants, will require only surgery.
In addition to her work for the Wilms' study group, Perlman has received more
than $1 million in funding from the National Institutes of Health to study gene
expression in Wilms tumor. Using a new technology called microchip gene array,
Perlman will measure the level of particular genes in Wilms tumors to identify
the 10 percent that are most aggressive. The study may lead to more effective
treatments for these tumors.
[Note: This article appeared in the Fall 2002 issue of Carousel, the magazine
of Children's Memorial Hospital.]