Testicular Tumors in Patients with Exstrophy-Epispadias Complex

Abstract

Due to separated pubic bone and patent processus vaginalis, males with exstrophy-epispadias complex often present with inguinal hernia during infancy. Since most of these testicles are operatively repositioned, testicular development is assumed to be normal. However, there is a paucity of knowledge about long-term testicular development in males with exstrophy-epispadias complex. We identified males with sonographic intratesticular abnormalities or testicular tumor in exstrophy-epispadias complex. Since 2003, a Germany wide cross-sectional followup study has been permanently offered to men with exstrophy-epispadias complex, focusing on andrological issues. A total of 22 men with exstrophy-epispadias complex presented to our clinical service for andrological evaluation, including testicular ultrasound. Sonography showed testicular and epididymal pathology in more than 50% of patients, with intratesticular abnormality in 23%, most commonly testicular microlithiasis (9%). Three patients underwent testicular biopsy. Histopathological evaluation revealed 1 case of testicular intraepithelial neoplasia and 2 benign testicular stromal tumors (1 Sertoli cell tumor and 1 Leydig cell tumor). Followup visits at 10, 28 and 68 months were uneventful. The observation of comorbid testicular tumor in males with exstrophy-epispadias complex should prompt a preventive health examination after puberty, which gives these patients the opportunity for further appropriate diagnostics and treatment if necessary. Biopsy is recommended for sonographically detected intratesticular lesions. Organ sparing procedures are worth considering, especially when stromal tumors with favorable outcome are discovered. However, current oncologic principles must be strictly followed. Although the etiology and true incidence of testicular tumors in exstrophy-epispadias complex are still unclear, our findings highlight the importance of long-term followup in patients with exstrophy-epispadias complex.