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True Hermaphroditism (Ovotesticular DSD)

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Clues in the Diagnosis of Non-tumoral Testicular Pathology

Abstract

The diagnosis of ovotesticular disorders of sexual differentiation (DSD) is made on a histological basis after noticing that the patient has both ovarian and testicular tissue. Karyotypes associated with this entity are varied, 46,XX being the commonest one. The explanation of the presence of testis alone or of the formation of ovotestes lends to several pathogenic interpretations. The phenotype varies from male to female without in many cases a correlation between genotype and phenotype. The ovotestis is the most frequent gonad, and in half of the patients, it is lodged in the abdominal cavity. Patients have multiple problems ranging from the assignment of one sex to the decision to retain one gonad or the other in order to protect its development, preserve their potential fertility, and lower the risk of a germ cell tumor. A summary of the most important data on the following topics is given in this chapter: karyotype, pathogenetic theories, phenotype, gonadal types, types of true hermaphroditism, biological behavior of the gonads, and patient management.

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Authors and Affiliations

  1. Universidad Autónoma de Madrid (UAM), Department of Anatomy, Histology and Neuroscience, School of Medicine, Madrid, Spain

    Manuel Nistal

  2. School of Medicine, Universidad Autónoma de Madrid (UAM), University Hospital La Paz, Department of Pathology, Madrid, Spain

    Pilar González-Peramato

  3. University Hospital Clinico San Carlos, Department of Urology, Madrid, Spain

    Álvaro Serrano

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  1. Manuel Nistal
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  2. Pilar González-Peramato
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  3. Álvaro Serrano
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Correspondence to Manuel Nistal .

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Nistal, M., González-Peramato, P., Serrano, Á. (2017). True Hermaphroditism (Ovotesticular DSD). In: Clues in the Diagnosis of Non-tumoral Testicular Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-49364-0_5

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  • DOI: https://doi.org/10.1007/978-3-319-49364-0_5

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  • Publisher Name: Springer, Cham

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  • Online ISBN: 978-3-319-49364-0

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