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Birth defects in three common pediatric malignancies; Wilms' tumor, neuroblastoma and Ewing's sarcoma
Insights
Congenital abnormalities are present in 29% of Wilms' tumor patients and 9% of neuroblastoma patients, but not in Ewing's sarcoma. These defects do not significantly impact survival rates for these pediatric cancers.
Area of Science:
- Pediatric Oncology
- Clinical Genetics
- Cancer Epidemiology
Background:
- Congenital abnormalities are known to be associated with certain childhood cancers.
- Understanding the prevalence and types of these abnormalities is crucial for patient management and prognosis.
- Previous studies have explored links between specific pediatric malignancies and congenital defects.
Purpose of the Study:
- To evaluate the incidence of congenital abnormalities in patients diagnosed with Wilms' tumor, neuroblastoma, and Ewing's sarcoma.
- To characterize the types of congenital defects observed in these pediatric cancer populations.
- To assess the impact of congenital abnormalities on the clinical presentation and survival outcomes of affected children.
Main Methods:
- Retrospective review of 84 pediatric cancer patients treated between 1965 and 1980.
- Systematic evaluation for the presence of congenital abnormalities at the time of diagnosis.
- Analysis of demographic data, defect types, age at diagnosis, and survival rates.
Main Results:
- Congenital abnormalities were identified in 29% of Wilms' tumor patients (10/34) and 9% of neuroblastoma patients (3/32), but in 0% of Ewing's sarcoma patients (0/18).
- In Wilms' tumor, common defects included genitourinary (10), central nervous system (3), and other structural anomalies (7).
- Congenital abnormalities did not significantly alter the median age at diagnosis or long-term survival rates for either Wilms' tumor or neuroblastoma patients.
Conclusions:
- Congenital abnormalities are relatively common in pediatric Wilms' tumor and neuroblastoma but rare in Ewing's sarcoma.
- The presence of congenital defects does not appear to adversely affect survival outcomes in these pediatric malignancies.
- Further research may elucidate specific genetic or environmental factors contributing to the co-occurrence of these conditions.
Abstract:
During the period 1965-1980, 84 patients with Wilms' tumor, neuroblastoma and Ewing's sarcoma were treated at the University of Rochester Medical Center. All patients were evaluated for the presence of congenital abnormalities. Ten of 34 (29%) patients with Wilms' tumor, 3 of 32 (9%) patients with neuroblastoma, and 0 of 18 patients with Ewing's sarcoma were so effected. In the patients with Wilms' tumor, 5 children had more than one abnormality. In this group, types of defects included genitourinary in 10 patients, central nervous system in 3, and other structural defects in 7. In the patients with neuroblastoma, the abnormalities were dissimilar. As expected, most of the 13 patients with congenital malformations were detected as having defects prior to the diagnosis of malignancy. For the patients with Wilms' tumor, median age at diagnosis for the entire group and for the subgroup with defects was 3 years, with age ranges similar. Male to female ratios were 1.3:1 and 1:1, respectively. Seventy percent of each group survived more than 5 years. For the patients with neuroblastoma, median age (range) for the entire group was 16 months (0-12 years). The patients with defects had ages of 5 days, 1 month and 9 months at diagnosis. Male to female ratio for the entire group was 0.6:1 and survival was 63% at 3 years (range 3-23 years). Those with defects are alive 3-8 years from diagnosis.