gonadoblastoma/femminilizzazione

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Role of gonadal dysgenesis in gonadoblastoma induction in 46, XY individuals. The Leuven experience in 46, XY pure gonadal dysgenesis and testicular feminization syndromes.

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To stress the importance of gonadal dysgenesis in the genesis of gonadoblastoma in the presence of the Y-chromosome, the authors report their experience on 7 patients with 46, XY Pure Gonadal Dysgenesis (PGD) and 14 patients with complete or incomplete forms of Testicular Feminization (TF) syndrome.

Feminization in gonadal dysgenesis associated with ovarian gonadoblastoma.

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A case of gonadal dysgenesis in a phenotypic female with XY genotype associated with ovarian gonadoblastoma is reported. Endocrinologic studies suggest that the normal female secondary sex characteristics could be due to estrogen production from luteal intersitial cells.

Dysgerminomas (seminomas) in genetic males with female phenotype. One case of gonadal dysgenesis and gonadoblastoma and one of testicular feminization.

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Gonadal tumors in patients with somatosexual ambiguity are rare but of considerable clinical and pathological interest. Two patients with dysgerminomas and abnormal sexual differentiation were recently treated at the Department of Gynecologic Oncology. One (Case 1) was an 18-year old girl and the

[The testicular feminization syndrome--a report of 9 cases].

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We present the data of analysis of 9 cases (girls and young women) aged 14 to 20 years, with syndrome of testicular feminisation. The patients were diagnosed and treated at the departments of Paediatric and Adolescent Gynaecology and of Gynaecological Endocrinology at the University Ob. & Gyn.

TSPY expression is variably altered in transgenic mice with testicular feminization.

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TSPY (testis-specific protein, Y-encoded) genes are expressed in premeiotic germ cells and round spermatids. The topology and timing of TSPY expression, and also its homology to members of the TTSN-family, suggest that TSPY is a proliferation factor for germ cells. There is also evidence for a role

Gonadal stromal tumors, gonadoblastomas, epidermoid cysts, and secondary tumors of the testis in children.

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Gonadal stromal tumors generally are benign and hormonally silent. When hormonally active, they may cause either virilization or feminization, posing an endocrine threat to the prepubertal boy. Accurate diagnosis is essential to differentiate those tumors that require medical management from those

Management of phenotypic female patients with an XY karyotype.

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Nine phenotypic female patients with XY karyotype were evaluated through a clinical, cytogenetic, hormonal, endoscopic and histologic diagnostic protocol. Seven patients complained of primary amenorrhea and two patients of abnormal puberal development. The final diagnosis was XY gonadal dysgenesis

[From embryogenesis to female phenotype in subjects with XY karyotype].

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On the basis of the findings of their investigation of the embryogenesis of the female genital tract, the authors advance a hypothesis concerning the origin of ambiguities between the genetic sex and the gonophoric sex which determine primary sexual characteristics observed in Morris' syndrome and

Male pseudohermaphroditism: genetics and clinical delineation.

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The genetics and clinical delineation of male pseudohermaphroditism are reviewed. These disorders are categorized initially by their genetic etiology--cytogenetic, Mendelian, or teratogenic. It is especially important to distinguish cytogenetic forms, usually associated with 45,X/46,XY mosaicism,

Testicular intratubular germ cell neoplasia in children and adolescents with intersex.

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In a review of 102 cases with various intersex states, the frequency of intratubular germ cell neoplasia (IGCN) unclassified in testes of children and adolescents was 6%. It was seen in 2 of 87 cases in the prepubertal age group and 4 of 23 cases in the pubertal age group. The frequency of IGCN was

Abnormal sexual differentiation and neoplasia.

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The prevalence of neoplasia is increased in individuals with certain disorders of sexual differentiation. Etiology and frequency of neoplasia vary with the particular disorder. In uncomplicated cryptorchidism, the testis is at least 10 times more likely to undergo neoplastic transformation than a

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