Neoplasms of dysgenetic gonads

Taylor, Herbert B.; Barter, Robert H.; Jacobson, Cecil B.

doi:10.1016/0002-9378(66)90677-6

Cited by 52 publications

(10 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Hence, the diagnosis of pure or partial gonadal dysgenesis can be reliably made, and surgical removal of the gonads should be considered, owing to increased risk of tumor development (23). Serum AMH assay proves to be extremely useful for a differential diagnosis, because AMH concentration is normal or high in Leydig cell aplasia or steroidogenic enzyme defects but is low or undetectable when the intersex condition is caused by gonadal dysgenesis.…”

Section: Discussionmentioning

confidence: 99%

Evaluation of Gonadal Function in 107 Intersex Patients by Means of Serum Antimüllerian Hormone Measurement¹

Rey

Belville

Nihoul‐Feketé

et al. 1999

The Journal of Clinical Endocrinology & Metabolism

View full text Add to dashboard Cite

Fetal male sexual differentiation is driven by two testicular hormones: testosterone (synthesized by interstitial Leydig cells) and antimüllerian hormone (AMH; produced by Sertoli cells present in the seminiferous tubules). Intersex states result either from gonadal dysgenesis, in which both Leydig and Sertoli cell populations are affected, or from impaired secretion or action of either testosterone or AMH. Until now, only Leydig cell function has been assessed in children with ambiguous genitalia, by means of testosterone assay. To determine whether serum AMH would help in the diagnosis of intersex conditions, we assayed serum AMH levels in 107 patients with ambiguous genitalia of various etiologies. In XY patients, AMH was low when the intersex condition was caused by abnormal testicular determination (including pure and partial gonadal dysgenesis) but was normal or elevated in patients with impaired testosterone secretion, whereas serum testosterone was low in both groups. AMH was also elevated during the first year of life and at puberty in intersex states caused by androgen insensitivity. In 46,XX patients with a normal male phenotype or ambiguous genitalia, in whom the diagnosis of female pseudohermaphroditism had been excluded, serum AMH levels higher than 75 pmol/L were indicative of the presence of testicular tissue and correlated with the mass of functional testicular parenchyma. In conclusion, serum AMH determination is a powerful tool to assess Sertoli cell function in children with intersex states, and it helps to distinguish between defects of male sexual differentiation caused by abnormal testicular determination and those resulting from isolated impairment of testosterone secretion or action.

show abstract

Section: Discussionmentioning

confidence: 99%

Evaluation of Gonadal Function in 107 Intersex Patients by Means of Serum Antimüllerian Hormone Measurement¹

Rey

Belville

Nihoul‐Feketé

et al. 1999

The Journal of Clinical Endocrinology & Metabolism

View full text Add to dashboard Cite

show abstract

“…Previously thought these patients do not seem to have an increased incidence of gonadal tumors [10]. But modern studies show patients with 46, XY pure gonadal dysgenesis are at a higher risk of developing gonadoblastoma and dysgerminoma, and may occur even in young ages.…”

Section: Discussionmentioning

confidence: 97%

Dysgerminoma in a case of 46, XY pure gonadal dysgenesis (swyer syndrome): a case report

Han

Wang

et al. 2011

Diagn Pathol

View full text Add to dashboard Cite

Simple 46, XY gonadal dysgenesis syndrome, also called Swyer syndrome, is known as pure gonadal dysgenesis. Individuals with the syndrome are characterized by 46, XY karyotype and phenotypically female with female genital appearance, normal Müllerian structures and absent testicular tissue. The condition usually first becomes apparent in adolescence with delayed puberty and primary amenorrhea due to the gonads have no hormonal or reproductive potential. Herein, we report a case of dysgerminoma diagnosed in a dysgenetic gonad of a 21-year-old patient with Swyer syndrome.

show abstract

“…Some dysgerminomas are associated with congenital abnormalities of the genital tract. These tumours may, however, need re-assessing in the light of recent work on intersexes (FATHALLA, RASHAD and KERR, 1966) and dysgenetic gonads (TAYLOR, BARTER and JACOBSON, 1966).…”

Section: Dysgerminoma and Gonadoblastomamentioning

confidence: 94%

Teratomas and Rare Nephrogenital Tumours

Brown¹,

Langley²

1968

Tumours in Children

View full text Add to dashboard Cite

Neoplasms of dysgenetic gonads

Cited by 52 publications

References 18 publications

Evaluation of Gonadal Function in 107 Intersex Patients by Means of Serum Antimüllerian Hormone Measurement¹

Evaluation of Gonadal Function in 107 Intersex Patients by Means of Serum Antimüllerian Hormone Measurement¹

Dysgerminoma in a case of 46, XY pure gonadal dysgenesis (swyer syndrome): a case report

Teratomas and Rare Nephrogenital Tumours

Contact Info

Product

Resources

About

Neoplasms of dysgenetic gonads

Cited by 52 publications

References 18 publications

Evaluation of Gonadal Function in 107 Intersex Patients by Means of Serum Antimüllerian Hormone Measurement1

Evaluation of Gonadal Function in 107 Intersex Patients by Means of Serum Antimüllerian Hormone Measurement1

Dysgerminoma in a case of 46, XY pure gonadal dysgenesis (swyer syndrome): a case report

Teratomas and Rare Nephrogenital Tumours

Contact Info

Product

Resources

About

Evaluation of Gonadal Function in 107 Intersex Patients by Means of Serum Antimüllerian Hormone Measurement¹

Evaluation of Gonadal Function in 107 Intersex Patients by Means of Serum Antimüllerian Hormone Measurement¹