Report of a rare case of true hermaphroditism in a 26-year-old woman

Articles in Press

Document Type : Case report

Authors

1 Department of Pathology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.

2 Department of Obstetrics and Gynecology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran

10.22038/ijogi.2025.86504.6373

Abstract

Introduction: True hermaphroditism is characterized by a patient's simultaneous presence of testicular and ovarian tissues. This case report describes a case of ovotesticular disorder in a 26-year-old woman.

Patient presentation: A 6-month-old child with ambiguous genitalia, including the simultaneous presence of a penis-like protrusion and a vagina, was referred to the clinic by her parents, and due to the karyotype 46, XX, surgical removal of the protrusion was performed. After puberty, the patient had a normal uterus, ovaries, breasts, menstrual cycle, and no beard or hoarseness, so she did not have any signs of male gender on clinical examination. Due to her masculine spirit, the patient underwent sex reassignment surgery at the age of 26 and underwent hysterectomy and salpingo-oophorectomy. Approximately 4 months later, a mastectomy was also performed. The macroscopic and microscopic features of the uterus were normal. However, in histopathology, a mixture of testicular and ovarian tissue was observed in the right gonad, and ovotesticular disorder was diagnosed.

Conclusion: The presence of a normal and female karyotype in childhood led to the failure to diagnose the disease for the studied individual, whereas considering ovotesticular disorder for the studied patient and performing diagnostic and therapeutic procedures in the early years of life could have been effectively improved the patient's quality of life. At the same time, since ototesticular disorder is a rare and complex abnormality with few reports, it is important to establish a systematic guideline for the evaluation and treatment of these patients in the country.

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The Iranian Journal of Obstetrics, Gynecology and Infertility

Articles in Press, Accepted Manuscript
Available Online from 22 June 2025

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