The History of Multimodal Treatment of Wilms' Tumor.

Multimodal therapy-surgery, radiation therapy, and chemotherapy-the foundation of modern cancer treatment, has led to dramatic improvements in survival. How the three disciplines coalesced to conquer Wilms' tumor is a compelling story that includes two of history's greatest discoveries, X-rays and antibiotics. By the mid-20th century both fields had matured to where dedicated clinicians and creative scientists could apply them to Wilms' tumor and achieve successive improvements in survival. William Ladd was able to achieve a zero operative mortality by 1940, but was left with a 32 per cent survival with surgery alone. Robert Gross and Edwin Neuhauser combined surgery and radiotherapy and achieve 47 per cent survival rate in 1950. Sidney Farber and his colleagues added an antibiotic, dactinomycin, to the treatment regimen and reached 80 per cent survival rate in 1966. The National Wilms' Tumor Study, organized in 1968, was a multidisciplinary effort of surgeons, radiotherapists, and pediatric oncologists across the country. By the 1990s, the National Wilms' Tumor Study achieved survival rates above 95 per cent while minimizing long-term effects through shortening courses of chemotherapy and radiation. The story of Wilms' tumor serves as a paragon for all types of cancer, in both children and adults.

Max Wilms and his tumor.

The most common cancer of the kidney in infants and children is named for Max Wilms, a German surgeon. How did this eponym come about? There were excellent reviews of this lesion before Wilms, a second year surgical assistant, published "Die Mischgeschwulste Der Niere" or The Mixed Tumors of the Kidney in 1899. At thirty two years of age, he demonstrated a masterful knowledge of pathology and embryology. Wilms' career was cut short when he became septic after operating on a prisoner of war during WWI. The survival rate for children with Wilms tumor was dismal until William Ladd, at the Boston Children's hospital introduced rational surgical treatment. By mid century, Robert Gross achieved a 47% survival rate with surgery combined with postoperative radiation. Sydney Farber treated Wilms tumors with Actinomycin-d and opened the door to cancer chemotherapy. With protocols developed by the National Wilms Tumor Study Group, the survival rate of children with Wilms tumors reached 90% by the end of the twentieth century.

Tales from previous times: important eponyms in pediatric surgery.

The use of eponyms in medicine has a long and captivating tradition, which applies to pediatric surgery as well. Unfortunately, even though these eponyms are widely used, oftentimes the fascinating personalities and lives behind these names remain unrecognized or underappreciated, especially among residents, fellows and younger-generation pediatric surgeons. Therefore, in this article, we review 15 names that are frequently used as eponyms in modern pediatric surgery and hereby enlighten the personalities behind them. Given their particular frequency, we choose the following eponyms: Ramstedt pylorotomy, Nissen fundoplication, Murphy's sign and sequence, McBurney's sign and incision, Meckel's diverticulum, Kasai portoenterostomy, Ladd's procedure, Morgagni and Bochdalek hernia, Ravitch operation, Nuss procedure, Hirschsprung disease, Swenson pullthrough, Peña procedure and Wilms tumor. A detailed description of the historical importance of these personalities and their contribution to our field is given. Without the appropriate historical background, it is difficult for the current younger and next generation pediatric surgeons to grasp the full spectrum of the ongoing progress in our field. Therefore, our article conveys not only important insight into the past, but also provides young surgeons with an important historical perspective essential to understand the current and future developments in modern pediatric surgery.

The Perlman syndrome: familial renal dysplasia with Wilms tumor, fetal gigantism and multiple congenital anomalies. 1984.

INTRODUCTION: The ensuing paper by Professor Giovanni Neri and colleagues was originally published in 1984, American Journal of Medical Genetics 19:195­207. The original article described a new family with a condition that the authors designated as the Perlman syndrome. This disorder, while uncommon, is an important multiple congenital anomaly and dysplasia syndrome; the causative gene was recently identified. This paper is a seminal work and is graciously republished by Wiley-Blackwell in the Special Festschrift issue honoring Professor Neri. We describe a familial syndrome of renal dysplasia, Wilms tumor, hyperplasia of the endocrine pancreas, fetal gigantism, multiple congenital anomalies and mental retardation. This condition was previously described by Perlman et al. [1973, 1975] and we propose to call it the "Perlman syndrome." It appears to be transmitted as an autosomal recessive trait. The possible relationships between dysplasia, neoplasia and malformation are discussed.

Jessop and the Wilms' tumor.

The earliest specialists in the field of pediatric surgical oncology were the surgeons of 19th century Europe, particularly Billroth, Kocher, and Wilms. However, the first successful nephrectomy for a Wilms' tumor in a child was performed by a less famous name, Thomas Richard Jessop (1837-1903) at the Leeds General Infirmary on June 7, 1877. Jessop ultimately became Vice President of the Royal College of Surgeons of England and was a gifted teacher, being at the forefront in the development of medical education outside of London as first Professor of Surgery in Leeds. The clinical case history and operative procedure of this first successful nephrectomy for Wilms' tumor in a child is presented.

Old man river. The flow of pediatric oncology.

Progress in pediatric oncology has been made by gathering individual investigators and their work into even larger research groups. The authors liken this process to the way small waters coalesce to form large rivers. This pattern can be traced back for most of the entities included under the rubric pediatric oncology, but it is more convenient and informative to do so with respect to two specific entities: acute lymphoblastic leukemia and Wilms' tumor. These malignancies are used as surrogates for the liquid and solid tumors and are addressed in this article.

[A century of progress in the knowledge and treatment of nephroblastoma]. / Un siècle de progrès dans la connaissance et le traitement du néphroblastome.

Since its initial description by Max Wilms over a century ago, nephroblastoma has benefited from considerable improvements both in terms of basic knowledge about it and management of it. Today, the majority of these very young patients can expect a long-term survival in excess of 90% at the price of a light therapy that combines surgical resection, chemotherapy based on ill-toxic agents, and in selected cases, radiotherapy of remarkably low toxicity. The contribution of large international studies will be emphasized here.

Historical aspects of the identification of the entity Wilms tumor, and its management.

Each era with its own ideas and contributions will be evaluated at some point by future generations. The main objective of this historical overview of Wilms tumor has been to document some of the accomplishments made in the past two centuries. Knowing from where researchers, investigators, physicians, and other caregivers in this field have come will undoubtedly help to place today's achievements as mere markers between the past and the future.

Pronephros and mesonephros--Cohnheim revisited.

Erroneous quotations in the literature and Cohnheim's statement, in his Lectures on General Pathology, that the Wolffian body or mesonephros is the first anlage of the urogenital system prompted this description of the growth of our knowledge of the early development of the kidney. Some of the pertinent older literature is reviewed, and the concept of the holonephros, as opposed to the trinephric view of kidney development, is discussed. Emphasis is placed on the decreasing functional significance of the pronephros with evolutionary development, to the extent that the role of the pronephros in the human is questioned. Cohnheim's seemingly erroneous reference to the development of the kidney is shown to be merely a reflection of the views current at his time.

Wilms--a man, a syndrome.

A biography is presented in commemoration of the 70th anniversary of the death of Max Wilms, whose name is almost exclusively known in association with the mixed tissue tumours of the kidney. Describing his curriculum vitae, we intended to place particular emphasis on the significance of his surgical work at the University of Heidelberg.