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Aniridia and Wilm's tumor.
Manisha Nada1, K N Rattan, Sarita Magu
1Department of Ophthalmology, Pt. B.D. Sharma PGIMS, Rohtak, Haryana, India.
Indian Journal of Pediatrics
|December 3, 2003
Summary
Aniridia, a rare eye disorder, is unusually common in children with Wilm's tumor. Early detection of sporadic aniridia can signal a heightened risk for this pediatric cancer.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Genetics
Background:
- Aniridia (absence of iris) is a rare congenital panocular disorder with a general population incidence of 1:50,000.
- A significantly higher prevalence of aniridia is observed in patients diagnosed with Wilm's tumor.
Observation:
- The study reviewed 60 Wilm's tumor cases, identifying aniridia in two patients.
- Both pediatric patients with Wilm's tumor and aniridia presented before the age of two years.
- The aniridia observed in these cases was sporadic.
Findings:
- Aniridia occurs in approximately 1 in 70 patients with Wilm's tumor, a rate vastly exceeding its general population frequency.
- Sporadic aniridia in children is associated with an early onset of Wilm's tumor.
Implications:
- Recognizing sporadic aniridia in a child should prompt heightened vigilance for potential Wilm's tumor development.
- This association underscores the importance of multidisciplinary evaluation for children presenting with aniridia.
- Early identification can lead to timely diagnosis and management of Wilm's tumor, potentially improving patient outcomes.