Pure seminoma arising in androgen insensitivity syndrome (testicular feminization syndrome): a case report and review of the literature

This is a case of a young woman who had testicular feminization syndrome and developed a seminoma in an undescended intrapelvic testis. Androgen insensitivity (testicular feminization) syndrome is a rare inherited form of male pseudohermaphroditism that occurs in phenotypically normal women with ade...

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Bibliographic Details
Published inModern pathology Vol. 6; no. 1; p. 89
Main Authors Collins, G M, Kim, D U, Logrono, R, Rickert, R R, Zablow, A, Breen, J L
Format Journal Article
LanguageEnglish
Published United States 01.01.1993
Subjects
Adult
Androgen-Insensitivity Syndrome - complications
Androgen-Insensitivity Syndrome - pathology
Cryptorchidism - pathology
Dysgerminoma - etiology
Dysgerminoma - pathology
Female
Humans
Male
Microscopy, Electron
Testicular Neoplasms - etiology
Testicular Neoplasms - pathology
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Summary:This is a case of a young woman who had testicular feminization syndrome and developed a seminoma in an undescended intrapelvic testis. Androgen insensitivity (testicular feminization) syndrome is a rare inherited form of male pseudohermaphroditism that occurs in phenotypically normal women with adequate breast development, normal external genitalia, a vagina of variable depth, absent uterus, and sparse or absent pubic and axillary hair. The gonad (undescended testes) may be intraabdominal, inguinal, or labial. These patients characteristically have male karyotype (XY) and negative sex chromatin and are at increased risk of undergoing malignant transformation of the undescended gonad. We review the literature emphasizing the biochemical and endocrinologic abnormalities leading to the syndrome, as well as the morphologic abnormalities (light microscopic) of the undescended testes, diagnosis, and therapeutic management.
ISSN:0893-3952