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External genital abnormalities associated with Wilms tumor.

M Beheshti, J F Mancer, B E Hardy

    Urology
    |August 1, 1984
    PubMed
    Summary

    Genital abnormalities are more common in children with Wilms tumor, especially bilateral cases. Male pseudohermaphroditism warrants monitoring for renal tumors.

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    Area of Science:

    • Pediatric Oncology
    • Urology
    • Genetics

    Background:

    • Wilms tumor is a common pediatric kidney cancer.
    • External genital abnormalities have been anecdotally linked to Wilms tumor.
    • Congenital mesoblastic nephroma is a rare infantile kidney tumor.

    Purpose of the Study:

    • To investigate the incidence of external genital abnormalities in patients with Wilms tumor and congenital mesoblastic nephroma.
    • To explore the association between genital anomalies and tumor characteristics, including laterality and histology.
    • To assess the risk of renal tumors in specific patient populations, such as those with male pseudohermaphroditism.

    Main Methods:

    • Retrospective review of medical records for 170 unilateral Wilms tumor, 18 bilateral Wilms tumor, and 6 congenital mesoblastic nephroma patients.
    • Analysis of the prevalence of external genital anomalies (cryptorchidism, hypospadias, gonadal dysgenesis, pseudohermaphroditism).
    • Comparison of anomaly rates between unilateral and bilateral Wilms tumor, and examination of associated tumor histology and patient demographics.

    Main Results:

    • Nine patients (Wilms tumor or congenital mesoblastic nephroma) presented with external genital anomalies.
    • External genital anomalies were significantly more frequent in bilateral Wilms tumor (27.7%) compared to unilateral Wilms tumor (2.3%).
    • Patients with genital anomalies often had unusual histologic subtypes and a high incidence of bilaterality; male pseudohermaphroditism showed a high incidence of Wilms tumors.

    Conclusions:

    • External genital abnormalities are associated with Wilms tumor at a higher than expected frequency, particularly in bilateral cases.
    • The co-occurrence of genital anomalies and Wilms tumor highlights specific tumor characteristics, including histology and bilaterality.
    • Male pseudohermaphrodite patients should be monitored for renal tumor development due to a noted increased incidence.

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