Study shows a new approach to treating patients with stage IV Wilms tumor

April 25, 2018, Children's National Medical Center

A new study showing significantly improved survival rates for patients with stage IV Wilms tumors with lung metastases was recently published in the Journal of Clinical Oncology. The outcomes of the study, "Treatment of Stage IV Favorable Histology Wilms Tumor With Lung Metastases: A Report From the Children's Oncology Group AREN0533 Study", will be a game-changer in treating Wilms tumor and reduce the need for radiation - and the long-term risks associated with it - in nearly half of patients whose cancer has spread to the lung. The study was led by Jeffrey Dome, M.D., Ph.D., vice president for the Center for Cancer and Blood Disorders at Children's National Health System.

Wilms tumor, which starts in the kidneys, is the fifth most common cancer in children under 15 years old. Although outcomes for most are outstanding, patients with metastatic disease, with the as the most common site of spread, fare worse than patients with localized disease. Their treatment is also complicated by a risk of health problems later in life, including cardiac dysfunction and second malignancies, which are most closely related to the use of lung radiation therapy (RT) and the chemotherapy drug doxorubicin.

The standard treatment for Wilms tumor with lung metastases in North America is to give "3-drug" chemotherapy (vincristine/dactinomycin/doxorubicin) and lung RT. The AREN0533 study assessed whether lung RT can be avoided for patients with complete lung nodule response after six weeks of chemotherapy. Conversely, the study assessed the benefit of adding two additional chemotherapy agents, cyclophosphamide and etoposide, to the treatment regimen for patients with incomplete lung nodule response, a treatment strategy that was associated with inferior outcomes in previous studies. AREN0533 was also the first study in Wilms tumor to modify treatment based on an adverse biological prognostic factor, loss of heterozygosity (LOH) at chromosomes 1p and 16q. The study showed:

  • The approach to therapy based on lung nodule response and LOH at chromosomes 1p and 16q resulted in a 4-year overall survival rate of 96 percent, compared to 84 percent on the predecessor study.
  • About 40 percent of patients with Wilms tumor and lung metastases can be spared initial upfront lung radiation and still have outstanding survival. This will decrease the long-term risk of heart toxicity and breast cancer.
  • Patients with incomplete lung nodule response after six weeks of therapy with cyclophosphamide and etoposide had significantly better 4-year event-free survival: 89 percent compared with 75 percent that was expected based on historical data.
  • Intensification of therapy for patients with LOH at 1p and 16q was highly effective: 4-year event-free survival rate improved from 66 percent on the previous study to 100 percent.

"These findings will change clinical practice and improve survival for patients with Wilms tumor whose cancer has spread to the lungs" said Dr. Dome. "The risk-adapted approach to treatment based on tumor biology and response provides a framework for future studies as we come one step closer to achieving 100 percent survival without -associated side effects."

Explore further: Is whole-brain radiation still best for brain metastases from small-cell lung cancer?

More information: David B. Dix et al, Treatment of Stage IV Favorable Histology Wilms Tumor With Lung Metastases: A Report From the Children's Oncology Group AREN0533 Study, Journal of Clinical Oncology (2018). DOI: 10.1200/JCO.2017.77.1931

Related Stories

Is whole-brain radiation still best for brain metastases from small-cell lung cancer?

April 16, 2018
Lung cancer often metastasizes to the brain. Historically, brain metastases have been treated with whole-brain radiation therapy. However, whole-brain radiation is associated with cognitive side-effects and studies of non-small ...

Radiation+immunotherapy can slow tumor growth for some patients with metastatic NSCLC

September 25, 2017
A new study involving patients with stage IV cancer finds that treatment with radiation therapy and immunotherapy can halt the growth of tumors by stimulating the body's immune system to attack the cancer. In the phase II ...

Chest radiation to treat childhood cancer increases patients' risk of breast cancer

October 27, 2014
A new study has found that patients who received chest radiation for Wilms tumor, a rare childhood cancer, face an increased risk of developing breast cancer later in life due to their radiation exposure. Published early ...

First-line immunotherapy treatment can improve survival for subset of lung cancer patients

June 21, 2017
Findings from a phase III clinical trial for advanced lung cancer patients could help oncologists better predict which patients are likely to receive the most benefit from immunotherapy as a first-line treatment based on ...

Adjuvant chemotherapy improves overall survival in patients with stage IB non-small cell lung cancer

June 13, 2016
The use of adjuvant chemotherapy in early-stage non-small cell lung cancer (NSCLC) patients improves overall survival (OS) and 5-year OS in patients with tumor sizes ranging from 3 - 7 cm.

Concurrent chemotherapy, proton therapy improves survival in patients with advanced lung cancer

July 20, 2017
For patients with advanced, inoperable stage 3 lung cancer, concurrent chemotherapy and the specialized radiation treatment, proton therapy, offers improved survival compared to historical data for standard of care, according ...

Recommended for you

Surgery unnecessary for many prostate cancer patients

December 13, 2018
Otherwise healthy men with advanced prostate cancer may benefit greatly from surgery, but many with this diagnosis have no need for it. These conclusions were reached by researchers after following a large group of Scandinavian ...

Combining three treatment strategies may significantly improve melanoma treatment

December 12, 2018
A study by a team led by a Massachusetts General Hospital (MGH) investigator finds evidence that combining three advanced treatment strategies for malignant melanoma—molecular targeted therapy, immune checkpoint blockade ...

An integrated approach to finding new treatments for breast cancer

December 12, 2018
Unraveling the complexity of cancer biology can lead to the identification new molecules involved in breast cancer and prompt new avenues for drug development. And proteogenomics, an integrated, multipronged approach, seems ...

New insight into stem cell behaviour highlights therapeutic target for cancer treatment

December 12, 2018
Research led by the University of Plymouth and Technische Universität Dresden has identified a new therapeutic target for cancer treatment and tissue regeneration – a protein called Prominin-1.

Lethal combination: Drug cocktail turns off the juice to cancer cells

December 12, 2018
A widely used diabetes medication combined with an antihypertensive drug specifically inhibits tumor growth—this was discovered by researchers from the University of Basel's Biozentrum two years ago. In a follow-up study, ...

Researchers use computer model to predict prostate cancer progression

December 12, 2018
An international team of cancer researchers from Denmark and Germany have used cancer patient data to develop a computer model that can predict the progression of prostate cancer. The model is currently being implemented ...

0 comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.