RESUMEN
Persistent Mullerian duct syndrome (PMDS) is a congenital disorder related to male sexual development. PMDS is usually diagnosed during an inguinal hernia cure. The diagnosis of PMDS following a testicular germ cell tumor is less common. We report the cases of three infertile male patients who were diagnosed with PMDS after surgery for germ cell tumors. They were 39, 27, and 37 years old men with a medical history of neglected cryptorchidism. All patients had a male karyotype and the ELISA test for the anti-Mullerian hormone was undetectable. Patients underwent chemotherapy followed by resection of residual mass in one patient. One patient is currently alive and disease-free. The two other patients died of systemic relapse. These cases highlight how early recognition and treatment of PMDS can prevent malignant germ cell tumors. The diagnosis of PMDS relies on a systemic assessment and analysis of mutations in the gene coding for AMH and AMHR-II. Key words: Persistent Müllerian duct syndrome (PMDS), anti mullerian hormone, germ cell neoplasm.
Asunto(s)
Neoplasias de Células Germinales y Embrionarias , Neoplasias Testiculares , Humanos , Masculino , Neoplasias de Células Germinales y Embrionarias/genética , Neoplasias Testiculares/genética , Progresión de la Enfermedad , Supervivencia sin EnfermedadRESUMEN
Leydig cell tumor (LCT) is a rare testicular tumor with a low incidence accounting 3% of all testicule neoplasms. Due to its rarity, the natural history of LCT is poorly understood. Patients can present with atypical symptoms and endocrine disorders. The diagnosis of LCT is based on histological and immunohistochemical examination. We report a new case of leydig cell tumor in a 61-year-old man presenting with a left testis hydrocele. The patient underwent a left orchidectomy and the diagnosis of LCT was established. Even in front of a benign pathology such as hydrocele, exploration is necessary to detect any testicle tumor.
