Disorders of Sexual Development, Male Pseudoermaphrodism

Nistal, Manuel; González-Peramato, Pilar

doi:10.1007/978-3-030-41894-6_4803

Manuel Nistal⁴ &
Pilar González-Peramato^5,6

Part of the book series: Encyclopedia of Pathology ((EP))

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Synonyms

Variant of mixed gonadal dysgenesis

Definition

It is a disorder of sexual development characterized by the presence of two dysgenetic testes or streak testis, frequently cryptorchid, associated with the presence of Müllerian derivatives, incomplete virilization, and in some cases Turnerian stigmata (Federman 1967).

Clinical Features

Incidence
Rare
Age
At any age
Sex
Male
Site
Both testes
Treatment
The risk of malignance is very high (46%), which justifies gonadectomy when the testes cannot be lowered and the genitals reconstructed.
Outcome
Patients to whom the testicles have been preserved require a periodic ultrasound follow-up. In case of bilateral orchiectomy, they would need supplemental hormone treatment.

Macroscopy

The gonad can be ovoid simulating a testis elongated like a streak testis

Microscopy

The gonads can be dysgenetic testis or streak testis (see “Disorder of Sex Development, Mixed Gonadal Dysgenesis”). In childhood, dysgenetic testis, apart from alterations of...

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References and Further Reading

Federman, D. D. (1967). Abnormal sexual development. Philadelphia: W.B. Saunders.
Google Scholar
Proença, E., Freitas, S., Fonseca, M., Figueiredo, S., & Rodrigues, C. (2001). Dysgenetic male pseudohermaphroditism. Acta Médica Portuguesa, 14, 511–514.
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Author information

Authors and Affiliations

Department of Anatomy, Histology and Neuroscience, School of Medicine, Universidad Autónoma de Madrid, Madrid, Spain
Manuel Nistal
Department of Pathology, Universidad Autónoma de Madrid, Madrid, Spain
Pilar González-Peramato
Department of Pathology, University Hospital La Paz, Madrid, Spain
Pilar González-Peramato

Authors

Manuel Nistal
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Pilar González-Peramato
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Corresponding author

Correspondence to Manuel Nistal .

Editor information

Editors and Affiliations

Histopathology and Molecular Diagnostics, University Hospital Careggi, Florence, Italy
Maria Rosaria Raspollini
Pathology Service, Champalimaud Clinical Center, Lisbon, Portugal
Antonio Lopez-Beltran
Unit of Anatomic Pathology, Department of Surgery, Cordoba University Medical School, Cordoba, Spain
Antonio Lopez-Beltran

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Nistal, M., González-Peramato, P. (2020). Disorders of Sexual Development, Male Pseudoermaphrodism. In: Raspollini, M.R., Lopez-Beltran, A. (eds) Uropathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-41894-6_4803

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DOI: https://doi.org/10.1007/978-3-030-41894-6_4803
Published: 19 June 2020
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-41893-9
Online ISBN: 978-3-030-41894-6
eBook Packages: MedicineReference Module Medicine

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