Objective: To present a case of successful pregnancy after conservative bilateral gonadectomy without adjuvant chemotherapy and radiotherapy in a 46,XY gonadal dysgenetic woman with gonadoblastoma and malignant germ cell tumor. Design: Case report. Setting: Infertility center in university hospital. Patient(s): An 18-year-old female with 46,XY karyotype underwent bilateral gonadectomy, and the pathology revealed gonadoblastoma with malignant mixed germ cell tumor. Intervention(s): Chromosomal analysis, bilateral gonadectomy, donor oocyte, and IVF program. Main Outcome Measure(s): Tumor markers and pregnancy result. Result(s): The patient was free from tumor recurrence after 13 years' follow-up. A successful twin pregnancy was achieved after oocyte donation and IVF. Conclusion(s): Gonadectomy without adjuvant therapies could be done in 46,XY gonadal dysgenetic women with gonadoblastoma in view of preservation of future fertility.
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