Nearly 1 in 4500 infants is born with abnormalities of the external genitalia. Contemporary management sees DSD decision-making as multifaceted, involving many different factors (none to the exclusion of the others), including etiology, fertility, and most likely gender outcome. Each patient needs to be considered in an individual basis. The surgeon plays an important initial role in the interdisciplinary group that an institution is obliged to have to take care of these complex patients. Evolution of practice in the last years tends to postpone surgery. Sex assignment does not mean inevitable surgical intervention.Except for gonadal biopsy or resection, no other surgery is performed in the neonatal period. Histology is only required for diagnosis in patients with abnormal gonads.If a streak gonad is recognized like in most patients with , it is removed without prior biopsy together with the surrounding peritoneum and the ipsilateral gonaduct . This gonad has to be removed, avoiding previous biopsy, as it has 25–50% chances to develop a gonadoblastoma and/or dysgerminoma and as there is the possibility of an in situ tumor at the time of the procedure.To analyze the spectrum of gonads of the DSD patients that we treated and assess the incidence of germ cell tumors, we conducted a prospective and observational study of DSD patients who underwent gonadal surgery. Age, sex assigned, scale of external masculinization (EMS), karyotype, molecular analysis, surgical approach, and pathology of the gonads were analyzed.We concluded that DSD patients with gonadal dysgenesis have a wide variability. The incidence of gonadoblastoma is not negligible in patients 46 XY and even feasible in 46 XX. The incidence of germ cell tumors (GCT) was 8.3, 6.6, and 16% in G1, 2, and 3, respectively. Early histological analysis and monitoring of these patients are mandatory.
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