Childhood Cancer

Wilms' Tumor

Wilms’ tumor is the most common form of childhood kidney cancer. It most often occurs in one kidney; although it can be found in both kidneys at the same time. 

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Latest Wilms' Tumor grants

George Q. Daley, MD/PhD, Principal Investigator
Boston Children's Hospital
Innovation Grants, Awarded 2016
James Amatruda, MD/PhD, Principal Investigator
University of Texas Southwestern Medical Center at Dallas
Innovation Grants, Awarded 2016
Kenneth Chen, MD, Principal Investigator
University of Texas Southwestern Medical Center at Dallas
Young Investigator Grants, Awarded 2016

Latest Wilms' Tumor blog posts

January 13, 2018

The first signs of Wilms’ tumor—a painless swelling in the abdomen, blood in the urine, belly pain, high blood pressure or fevers—often seem to be symptoms of something much less scary. A pediatrician will recommend an abdominal ultrasound and then a diagnosis will be made. Treatment, which typically includes surgery, radiation and chemotherapy, begins. Most children, even those with higher stages of the disease can finish treatment knowing that they will most likely never battle Wilms’ tumor again.

However, being cured of Wilms’ tumor can come with a bevy of long-term side effects—loss of kidney function, bone and skeletal deformities, lung issues and more cancer.

With each year of remission, the risk of a secondary cancer increase, particularly for those who require radiation.

Dr. Julie Glade-Bender

Dr. Julia Glade-Bender

“No matter what you do, you are exposing a fair amount of a child’s body to radiation and every little bit of radiation counts, particularly in children who have already declared themselves as a ‘tumor-former’,” said Dr. Julia Glade Bender, a member of the ALSF Scientific Advisory Board and an oncologist at New York-Presbyterian Hospital/Morgan Stanley Children’s Hospital.

Now, oncologists are studying the biological drivers of Wilms’ tumor to understand how to reach a 100% cure rate, while minimizing long-term side effects.

The cures and the side effects. 
For years, the mainstay of treatment was surgery, chemotherapy and aggressive radiation. Clinical trials have shown that chemotherapy can be shortened and radiation can be eliminated for many children with Wilms’ tumor. For those who still require it, doses of radiation have been reduced, but not enough to completely avoid potential significant long-term health risks.

Radiation can leave a child susceptible to skeletal deformities—the radiated side of the spine can grow slower than the non-radiated side. Radiation can also cause restrictive lung disease later in life and hamper an individual’s ability to breathe. It also exposes a child to the risk of more cancer.

Every cancer is different.
Under the microscope, Wilms’ tumor cells look similar to other childhood cancer cells—round blue embryonic cells.

However, the microscope does not tell the whole story.

“Every cancer is different, because every child is different,” said Dr. Glade Bender. “The critical question is: which cure goes with which patient?”

The biology of Wilms’ tumor coupled with the biology of individual patients affected by disease are two areas of specific interest to researchers. They are working to understand the origins of the disease and also to understand why some cases of Wilms’ tumor are treatment resistant.

Researchers are performing retrospective studies and reviewing past cases of children who relapsed following frontline treatment. They are also working to study patterns of chromosomal changes that happen in children diagnosed with Wilms’ tumor.

Dr. Glade Bender and other ALSF-funded researchers see promise in the study of developmental therapeutics, which tests and examines new treatment agents in children after standard therapy has failed. These studies have the potential to identify new drugs that can help children who relapse and provide meaningful insights into offering safer treatments from the very beginning to future children affected by cancer.

“We won’t stop searching for cures, until we are at 100%,” said Dr. Glade Bender.

Learn more about Wilms’ tumor research and ALSF-funded projects, here.

November 8, 2017

“I want chocolate milk, IMMEDIATELY,” those were 4-year-old Sophia’s first words following a seven-hour surgery to remove a tumor on her kidney. 

It is no surprise that little Sophia, who is now 8 years old and a survivor of Wilms’ tumor, the most common type of kidney cancer in children, describes herself  as “one tough cookie.”

Wilms’ tumor typically presents itself as a painless swelling of the belly. In Sophia’s case, her pediatrician noticed it at her routine 4-year-old checkup. For other children, a parent might notice the swelling or even, rarely, blood in their child’s urine.

Treatment includes surgery to remove the tumor or the entire affected kidney, chemotherapy and radiation. The disease has a high cure rate—as high as 90% for most children—but high doses of radiation can cause long-term side effects. Children who have been treated for Wilms’ tumor have regular scans to check for both relapse and side effects. 

Sophia had surgery, followed by 18 months of chemotherapy and radiation at Wesley Medical Center in Wichita, Kansas. Treatment was hard—but her family worked just as hard to keep Sophia’s spirits high with special outings and lots of love. Sophia kept her spunky, tough cookie attitude throughout treatment. 

Sophia has been three years cancer-free and, together with her family, is a Hero Ambassador for Alex’s Lemonade Stand Foundation. Her family knows how important childhood cancer research is for children like their daughter.

“I’m so grateful that Sophia is well and that there’s a treatment plan for her, but she suffered,” said Tiffany Stepien, Sophia’s mom. 

Treatment for Wilms’ tumor has been largely unchanged for some time, because of its high cure rate. However, high doses of radiation to a child can come with several serious side effects, including scoliosis, restrictive lung disease and sarcoma, later in life. Now, research is studying how targeted, individualized treatments can help limit the use of high dose chemotherapy and radiation, so children can be cured without being harmed. Research is also looking to identify the highest risk patients—those who may be prone to relapse—so that this group can receive higher doses of frontline treatment and beat Wilms’ tumor without relapsing. 

Sophia is already planning to help search for safer, more effective treatments for kids like her. 

“Sophia always said she’s going to invent a chemo that only gets the cancer cells and not hair or stomach cells,” said Tiffany. “And then, “I am going to invent glasses that can just look at my belly and know the cancer is gone and I won’t need a CT scan.”

Read more about Sophia here.

ALSF funds several research projects studying Wilms’ tumor, as well as Beckwith-Wiedemann Syndrome, a genetic predisposition disorder that can lead to the development of Wilms’ tumor. Read more about that research here.