Historical Background of Wilms Tumor

 

By Max J. Coppes, MD, PhD, MBA and Arty R. Coppes, MA

 

The eponym Wilms tumor is well known to pediatric oncologists all over the world and refers to the most common form of renal cancer in the pediatric age group. Its occurrence in adults is rare, but not unheard of. Wilms tumor, also referred to as nephroblastoma, is named after the German surgeon Max Wilms, who was born Nov. 5, 1867 in Hünshoven near Aachen, Germany. 

 

After obtaining his medical degree from the Rheinische Friedrich-Wilhelm Universitåt Bonn in Germany in 1891, the young Wilms was attracted to surgery. In a move that would raise many eyebrows in today’s culture of focusing narrowly and if possible, early on, Wilms deliberately sought to expand his experience before going into surgery. He successfully applied to work as a pathologist for four years under Eugen Bostroem at the Pathologisch-Anatomischen Institut in Giessen. As fate would have it, it was during these years that he saw his first nephroblastoma that would result in his famous 1899 monograph Die Mischgeschwülste der Niere.

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Dr. Carl Max Wilhelm Wilms (1867-1918)

 

As has been reported before, Wilms was not the first one to describe what later became known as Wilms tumor. Review of the literature suggests that Thomas F. Rance in 1814 may have been the first to describe this pediatric tumor, although Rance’s histologic description is not very specific and could indeed have represented another pediatric tumor of the kidney. Doubt about the diagnosis of Wilms tumor does not exist for Carl Joseph Eberth’s 1872 manuscript on a young patient with bilateral renal tumors. 

 

Although Wilms was definitely not the first one to describe the tumor, he provided such a comprehensive review of pediatric renal tumors (adding a few new cases from his own experience) that the disease entity that bears his name became recognized as a separate entity. This eponym exists despite the fact that five years prior to Wilms’ manuscript the German pathologist Felix V. Birch-Hirschfeld and his countryman and gynecologist/obstetrician Albert Siegmund Gustav Döderlein reported that they believed that a distinct pediatric renal cancer type had previously been described. They believed the cancer type had been described using a number of different terms, such as mesoblastic sarcoma, embryonal sarcoma, adenosarcoma and nephrogenous dysembryoma. 

 

For a brief period, what we now refer to as Wilms tumor was known as Birch-Hirschfeld tumor in the German literature. For unknown reasons, Wilms manuscript was noted more broadly in the literature than that of Birch-Hirschfeld and Döderlein and eventually resulted in the eponym that carries his name. Wilms recognized that all tissues seen in nephroblastoma develop from cells of the mesoderm “similar to the growth of an embryo, all these tissues develop from a common and macroscopically undifferentiated germ cell.” 

 

After four years in Giessen, Wilms briefly joined the department of internal medicine in Köln as a pathologist before starting his formal surgical training in 1897 with the world-renowned Friedrich Adolf Trendelenburg in Leipzig. During his early years as a surgeon, Wilms wrote a textbook entitled Der Ileus: Pathologie und Klinik des Darmverschlusses, a book on the surgical aspects of intestinal obstruction. Oddly, until then, intestinal obstruction had been viewed as a medical, not surgical, problem. His contribution became a landmark in medical literature and established him as a leading surgeon. 

 

His first position of chief of a surgical service was in Basel, Switzerland. Shortly afterward, in 1910, he was given the opportunity to succeed professor Albert Naraths as chairman of the very prestigious department of surgery in Heidelberg. He accepted and would remain in Heidelberg until his untimely death caused by diphtheria in 1918. While performing emergency surgery on a French P.O.W. who had a swollen larynx associated with diphtheria, Wilms became infected with the disease, and died within a few days at the age of 50. Reportedly, he was successful in saving the life of the French soldier. After his death, his position at Heidelberg was filled by surgeon Eugen Enderlen (1863–1940).

 

Aside from the manuscript that gave nephroblastoma its currently favored name, Wilms received many honours and contributed to medicine with several innovations. Among his contributions was the design of a tendon suture technique, known in German literature as the “Wilms-Sieverischen” suture. He also developed a spinal cord pressure mercury manometer that was extensively used during World War I and an X-ray exam table that prevented superposition of the spine over the esophagus. In so doing, his table provided a better way to visualize and examine the esophagus. 

Major Medical Milestones in the History of Wilms Tumor

 

The history of Wilms tumor reflects more than a century of medical progress, transforming a disease that was once almost universally fatal into one of the most curable forms of childhood cancer. The following milestones highlight some of the most important discoveries, treatments, and breakthroughs that have shaped modern Wilms tumor care.

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1899 – Dr. Max Wilms Describes the Disease

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German surgeon and pathologist Dr. Max Wilms published the first comprehensive description of pediatric kidney tumors in children.

His work identified the disease as a distinct form of childhood kidney cancer and laid the foundation for future research. The disease later became known as Wilms tumor in recognition of his contributions.

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Early 1900s – Surgery Becomes the Primary Treatment

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During the early twentieth century, surgery was the only available treatment option.

Children diagnosed with Wilms tumor often underwent nephrectomy (kidney removal), but survival rates remained extremely poor because cancer frequently spread before diagnosis or recurred after surgery.

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Survival rates during this period were generally below 20%.

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1950s – Introduction of Radiation Therapy

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The introduction of radiation therapy represented one of the first major advances in treatment.

Doctors discovered that combining surgery with radiation therapy could improve local tumor control and reduce recurrence rates.

This marked the beginning of multimodal treatment approaches for childhood cancer.

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1960s – Discovery of Effective Chemotherapy

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The development of chemotherapy dramatically changed outcomes for children with Wilms tumor.

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Researchers found that drugs such as:

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• Actinomycin D (Dactinomycin)

• Vincristine

 

could significantly improve survival when used alongside surgery and radiation therapy.

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For the first time, large numbers of children began surviving the disease.

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1969 – Formation of the National Wilms Tumor Study Group (NWTS)

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One of the most important milestones in Wilms tumor history was the creation of the National Wilms Tumor Study Group (NWTS) in North America.

This collaborative research network brought together hospitals and researchers to conduct large clinical trials aimed at improving treatment outcomes.

The NWTS helped establish many of the treatment protocols still used today.

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1970s – Rapid Improvements in Survival

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Throughout the 1970s, researchers refined treatment protocols using combinations of:

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• Surgery

• Chemotherapy

• Radiation therapy

 

These advances resulted in dramatic improvements in survival rates and helped establish Wilms tumor as one of the great success stories in pediatric oncology.

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1980s – Improved Tumor Staging and Risk Classification

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Doctors developed more accurate staging systems to classify Wilms tumors according to:

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• Tumor size

• Spread of disease

• Histology (tumor appearance under a microscope)

 

This allowed treatment to be tailored according to risk, reducing unnecessary treatment for lower-risk patients while intensifying therapy for more aggressive tumors.

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1990s – Histology-Based Treatment Approaches

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Researchers discovered that tumor histology was one of the strongest predictors of outcome.

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The distinction between:

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• Favorable histology Wilms tumor

• Unfavorable (anaplastic) histology Wilms tumor

 

became a critical component of treatment planning.

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Risk-adapted therapy further improved survival while reducing long-term side effects.

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1990s–2000s – Advanced Medical Imaging

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The widespread use of:

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• Ultrasound

• CT scans

• MRI imaging

 

improved the diagnosis, staging, and monitoring of Wilms tumor.

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Earlier and more accurate detection allowed doctors to better plan treatment and identify recurrence sooner.

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2000s – Genetic Discoveries

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Scientists identified several important genetic changes associated with Wilms tumor, including abnormalities involving:

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• WT1 gene

• WT2 region

• WTX gene

• CTNNB1 gene

 

These discoveries improved understanding of how the disease develops and opened new areas of research into tumor biology and targeted treatments.

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2000s–2010s – International Treatment Collaboration

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Two major international treatment approaches became established:

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Children's Oncology Group (COG)

Primarily used in North America.

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International Society of Paediatric Oncology (SIOP)

Used across Europe and many other parts of the world.

Large international clinical trials helped refine treatment protocols and improve outcomes globally.

 

2010s – Focus on Reducing Long-Term Side Effects

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As survival rates improved, researchers increasingly focused on survivorship and quality of life.

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Studies examined long-term effects involving:

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• Kidney function

• Fertility

• Heart health

• Growth and development

• Secondary cancers

• Emotional wellbeing

 

The goal shifted from simply curing children to helping them thrive throughout adulthood.

 

2020s – Precision Medicine and Risk-Based Treatment

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Modern research focuses on:

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• Molecular biology

• Tumor genetics

• Biomarker discovery

• Precision medicine

• Reduced toxicity treatments

 

Researchers are working to identify which children require intensive treatment and which can safely receive less therapy while maintaining excellent outcomes.

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Today – One of the Greatest Success Stories in Childhood Cancer

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More than 125 years after Dr. Max Wilms first described the disease, Wilms tumor is now considered one of the most treatable childhood cancers.

In many developed healthcare systems:

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• Overall survival exceeds 90%

• Early-stage survival rates are often above 95%

• Treatment protocols continue to improve

• Long-term survivorship care is becoming increasingly sophisticated

 

Despite this progress, significant global disparities remain. The next chapter in the history of Wilms tumor will likely focus on ensuring that children everywhere- regardless of where they live - can benefit from the medical advances achieved over the past century.

Frequently Asked Questions (FAQs)

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About the History of Wilms Tumor

 

Who was Dr. Max Wilms and why was Wilms tumor named after him?

Wilms tumor is named after Dr. Max Wilms, a German surgeon and pathologist who published the first detailed description of the disease in 1899. His pioneering research helped establish Wilms tumor as a distinct form of childhood kidney cancer and laid the foundation for future diagnosis, treatment, and research.

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When was Wilms tumor first discovered and who discovered it?

Wilms tumor was first comprehensively described in 1899 by Dr. Max Wilms. Although childhood kidney tumors had been observed before this time, Dr. Wilms was the first physician to systematically study and classify the disease.

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What was Wilms tumor originally called?

Before becoming widely known as Wilms tumor, the disease was often referred to as nephroblastoma, a term that is still used today in medical literature. The name Wilms tumor was later adopted in recognition of Dr. Max Wilms' contributions to understanding childhood kidney cancer.

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How was Wilms tumor treated before chemotherapy was available?

Before chemotherapy was developed, treatment options for Wilms tumor were extremely limited and typically involved surgery alone. Survival rates were poor because many tumors had already spread before diagnosis and effective cancer medicines were not yet available.

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When did chemotherapy for Wilms tumor become available?

Chemotherapy became an important part of Wilms tumor treatment during the 1960s. The introduction of drugs such as vincristine and dactinomycin dramatically improved survival rates and helped transform Wilms tumor into one of the most treatable childhood cancers.

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How have survival rates for Wilms tumor changed over time?

Survival rates for Wilms tumor have improved dramatically over the past century. In the early 1900s, most children diagnosed with the disease did not survive. Today, survival rates exceed 90% in many developed healthcare systems thanks to advances in surgery, chemotherapy, radiation therapy, medical imaging, and supportive care.

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Why is Wilms tumor considered one of the greatest success stories in childhood cancer?

Wilms tumor is often regarded as one of the greatest success stories in pediatric oncology because survival rates have increased from less than 20% in the early twentieth century to more than 90% today. Few childhood cancers have experienced such dramatic improvements in outcomes over a relatively short period of medical history.

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What were the most important breakthroughs in the history of Wilms tumor treatment?

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Several medical advances transformed Wilms tumor treatment, including:

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• Development of nephrectomy surgery

• Introduction of radiation therapy

• Discovery of effective chemotherapy medicines

• Creation of international clinical trial groups

• Improved medical imaging

• Histology-based treatment planning

• Genetic and molecular research

• Modern survivorship programs

 

Each of these milestones helped improve survival rates and quality of life for children with childhood kidney cancer.

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What was the National Wilms Tumor Study Group and why was it important?

The National Wilms Tumor Study Group (NWTS), established in 1969, was one of the most influential pediatric cancer research collaborations in history. It helped develop many of the treatment protocols that significantly improved survival rates for children with Wilms tumor and continues to influence modern care.

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How did international research improve Wilms tumor survival rates?

International collaboration between organizations such as the Children's Oncology Group (COG) and the International Society of Paediatric Oncology (SIOP) helped standardize treatment protocols, improve clinical research, and identify the most effective therapies for children with Wilms tumor worldwide.

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What role has genetics played in understanding Wilms tumor?

Genetic research has helped scientists better understand how Wilms tumor develops and why some children are at increased risk. Important discoveries involving genes such as WT1 and WT2 have improved knowledge of childhood kidney cancer biology and may contribute to future treatment advances.

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How has Wilms tumor treatment changed over the last 100 years?

Over the last century, Wilms tumor treatment has evolved from surgery alone to highly specialized multidisciplinary care involving chemotherapy, radiation therapy, advanced imaging, stem cell transplant, fertility preservation, survivorship programs, and long-term follow-up care.

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What is the future of Wilms tumor research and treatment?

Researchers are currently focusing on precision medicine, tumor genetics, targeted therapies, reduced treatment toxicity, survivorship care, and earlier diagnosis strategies. Future advances aim to improve survival outcomes while reducing the long-term side effects associated with childhood cancer treatment.

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Why is the history of Wilms tumor important for families today?

Understanding the history of Wilms tumor helps families appreciate how far treatment has advanced and why survival rates are now so much higher than they were in the past. It also highlights the importance of ongoing research, early diagnosis, and global efforts to ensure that every child has access to effective treatment and care regardless of where they live.