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Congenital anomalies in children with Wilms' tumor: a new survey
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This study confirms that children with Wilms tumor often have aniridia and genitourinary abnormalities. It also suggests hemihypertrophy is increasingly recognized alongside Wilms tumor in pediatric cancer patients.
Area of Science:
- Pediatric Oncology
- Clinical Genetics
- Childhood Cancer Research
Background:
- Wilms tumor is a common pediatric kidney cancer.
- Certain congenital anomalies are known to be associated with Wilms tumor.
- Understanding these associations aids in diagnosis and management.
Purpose of the Study:
- To investigate the frequency of specific congenital anomalies in patients diagnosed with Wilms tumor.
- To examine the co-occurrence of aniridia, hemihypertrophy, and genitourinary abnormalities with Wilms tumor.
- To assess changes in the recognition of these associations over time.
Main Methods:
- Retrospective analysis of medical records from 547 patients with Wilms tumor across 68 hospitals.
- Data collection focused on the presence of aniridia, hemihypertrophy, and genitourinary abnormalities.
- Family history of Wilms tumor was also reviewed.
Main Results:
- Six patients (1.1%) had aniridia.
- 16 patients (2.9%) had hemihypertrophy.
- 24 patients (4.4%) had genitourinary abnormalities.
- Multiple Wilms tumor cases were identified in three families.
- The concurrence of hemihypertrophy with Wilms tumor appears to be more frequently recorded now.
Conclusions:
- Aniridia and genitourinary anomalies are confirmed as frequent co-occurring conditions in Wilms tumor patients.
- Hemihypertrophy recognition alongside Wilms tumor may be increasing.
- Checklists for other childhood neoplasms and their associated anomalies are recommended.
Abstract:
From 68 hospitals in the National Wilms' Tumor Study, records of 547 patients showed six patients with aniridia, 16 with hemihypertrophy, and 24 with genitourinary abnormalities. Multiple cases of Wilms' tumor occurred in three families. The results confirm high frequencies of aniridia and genitourinary anomalies in patients with Wilms' tumor, and show that concurrence with hemihypertrophy may be more often recognized or recorded now than it was ten years ago. The results demonstrate the desirability of developing checklists for other childhood neoplasms some of which have their own constellation of anomalies.