An update on the management of Wilms' tumour (original) (raw)
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Long-term kidney function in children with Wilms tumour and constitutional WT1 pathogenic variant
Pediatric Nephrology
Background Wilms tumour (WT) survivors, especially patients with associated syndromes or genitourinary anomalies due to constitutional WT1 pathogenic variant, have increased risk of kidney failure. We describe the long-term kidney function in children with WT and WT1 pathogenic variant to inform the surgical strategy and oncological management of such complex children. Methods Retrospective analysis of patients with WT and constitutional WT1 pathogenic variant treated at a single centre between 1993 and 2016, reviewing genotype, phenotype, tumour histology, laterality, treatment, patient survival, and kidney outcome. Results We identified 25 patients (60% male, median age at diagnosis 14 months, range 4–74 months) with WT1 deletion (4), missense (2), nonsense (8), frameshift (7), or splice site (4) pathogenic variant. Thirteen (52%) had bilateral disease, 3 (12%) had WT-aniridia, 1 had incomplete Denys-Drash syndrome, 11 (44%) had genitourinary malformation, and 10 (40%) had no phen...
Wilms' tumor and related abnormalities in the fetus and newborn
Seminars in Perinatology, 1999
Mesoblastic nephroma, a benign tumor, is the most common renal neoplasm in neonates. Wilms' tumor (WT) may occur in newborn infants, but is more common in older children. The molecular genetics of WT involves one or more genes located on Chromosome #11 and probably other locations not yet elicidated. Germline mutations cause less than 5% of WTs; most WTs are sporadic. Precursor lesions to WT called nephrogenic rests may be detected before evolution to WT by imaging studies. Developmental anomalies comprising several different syndromes are associated with nephrogenic rests and predisposition to WT. Prospective surveillance for WT may be feasible in high risk infants identified on the basis of physical findings followed by testing for predisposing gene defects and periodic imaging of the kidneys and other organs at risk until the period of risk has ended.
Case Report on Wilm’s Tumor in Children
Journal of Pharmaceutical Research International, 2021
Introduction: Wilms tumor is the most frequent kind of kidney cancer in kids. It is responsible for around 6% of all tumors in children. In children under the age of 15, the incidence is 7.6% cases per million, with 75% of cases occurring before the child reaches the age of five. A variant term for wilms tumor is nephroblastoma. It is most widespread between the ages of three and four, after which it becomes much less frequent. Clinical Findings: Wilms tumor in children produces one or more of the following signs and symptoms: Abdominal pain Increasing abdominal girth Lack of appetite Fever Blood in the urine Nausea or vomiting. Constipation Shortness of breath Diagnostic Evaluation: Increase abdominal girth, pain, fever, vomiting, cried at the time of urination, hematuria, black stool, pallor, hypertension and moderate microcytic anemia (Iron deficiency). Ultrasonography: Splenomegaly Therapeutic Investigation: X- ray of abdomen and chest, IVP, USG, MRI, CT scan, renal function te...
Wilms' Tumor of the Kidney: Insights into Risk Factors, Pathogenesis, Diagnosis and Management
Wilms' tumor is a rare disease of the kidney that usually affects newborn and young children. It is the commonest renal tumor of childhood affecting one in 10,000 children. It may spread into the other kidney if it has not been discovered early. Diagnosis depends on physical examination and radiographic images while the treatment decision is taken after determining the stage of the disease according to the occurrence of metastasis or the presence of the cancer cells in both kidneys. Lines of treatment include surgical excision, high dose chemotherapy and radiotherapy. Prognosis depends on the stage of the disease at diagnosis, tumor size, histopathological features of the tumor and the age of the patient.
A rare case of a Wilms tumor: case report
2018
The nephroblastoma or Wilms tumor (WT) is the most common renal tumor in childhood, representing approximately 6-7% of all pediatric cancers, with a yearly incidence of 10 cases in one million children less than 15 years old, and continues to arouse interest by remarkable actual therapeutic successes, consecutive to the multidisciplinary approach. Its maximum incidence is around the age of 3-3.5 years old, having an equal frequency in males and females. We present the case of a child, aged three years and five months, who was diagnosed with WT (nephroblastoma) with triphasic pattern, stage II tumor, and admitted to the Department of Oncopediatry for chemotherapeutic treatment and clinico-biological investigations.
Wilms' tumour - a case of disrupted development
Journal of Cell Science, 1994
Wilms' tumour is a paediatric kidney malignancy that arises through aberrant differentiation of nephric stem cells. We are studying the role of one Wilms' tumour pre disposition gene, WT1. This is a tumour suppressor gene whose function is required for normal development of the genitourinary system. WT1 encodes a putative transcrip tional repressor of the zinc finger family. Here we discuss how one of the normal functions of WT1 may be to suppress myogenesis during kidney development. Further-
Indian Journal of Urology, 2007
Wilms' tumor (WT) is the commonest pediatric renal tumor, predominantly seen in children less than five years of age. The majority of patients present with an abdominal lump and CT scan is the usual imaging modality for determining the extent of disease. With multimodality management, the results of treatment of WT have improved dramatically over the last 50 years. The treatment protocols have been devised and modified repeatedly depending on evidence from randomized trials by several cooperative groups-mainly National Wilms' Tumor Study Group (NWTSG) and the International Society of Pediatric Oncology (SIOP). The NWTSG recommends primary surgery followed by chemotherapy while SIOP advocates four weeks of chemotherapy prior to surgery. The regimen, dose and duration of chemotherapy have been repeatedly modified to reduce toxicity while maintaining efficacy. The role of radiation therapy has also been customized. Most centers have reported excellent survival rates with the modern day treatment protocols, except in patients with an unfavorable histology. The results of treatment of relapsed WT have also improved with newer drugs and combinations being used for the same.
Current management of Wilms’ tumor in children
Journal of Pediatric Urology, 2009
Wilms' tumor is the most common renal tumor in children. Outcomes have improved dramatically over the past few decades, but important treatment questions remain. These include the role of molecular biologic markers in stratifying patients for therapy or targeting tumors for treatment. We present a summary of these advances and outline the current treatment of Wilm's tumor. Materials and methods: The medical literature and results of all cooperative group studies reporting treatment of children with Wilms' tumor were reviewed. Results: Overall survival exceeds 90% for most patients with nephroblastoma. However, outcomes for patients with rhabdoid tumors and diffuse anaplasia remain poor. The role of renal sparing surgery in patients with bilateral tumors is clear, but for children with unilateral tumors it continues to be defined. Conclusions: Current protocols conducted by pediatric oncology groups are beginning to incorporate biologic features to stratify patients for therapy. Treatment strategies continue to focus on limiting late effects of treatment while maintaining an excellent survival. New therapies are needed to treat the high-risk patients who continue to have high relapse and mortality rates.