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- W1977976472 abstract "The risk of malignancy in dysgenetic gonads of patients with a Y chromosomal component seems to be significant. The age at onset of malignancy is unknown. Two cases with germ cell neoplasms are presented, both with normal S-alpha-fetoprotein levels. Case I: A 46, XY true hermaphrodite with ambiguous external genitalia, persistent Mullerian structures, a cryptorcide testis on the left and an ovary on the right side. At the age of 10 years a laparotomy was performed. The plasma levels of testosterone was 5.4 nmol/1, dihydrotestosterone 1.0 nmol/l, oeotradiol<40 pmol/l, LH 10 U/l, and FSH 28 U/l. The bilateral gonadectomy revealed gonadoblastoma and dysgerminomo in the ovary, and an epithelial a-typia in the testis. Case II: A 46, XY mixed gonadal dysgenesis with hypospadia and a lobio-scrotal fold, persistent Mullerian structures, and a testicular gonad on one side and no gonadal tissue on the other. At the age of 13 years plasma level of FSH was 4 U/l, LH 33 U/l, oestradiol<40 pmol/l, testosterone failed, but the urinary excretion of 17-KS was low for age (0.60 mg/24 h). At the subseguent gonadectomy a teratocarcinoma was revealed in the testis. Conclusion: Germ cell neoplasms appear before the age of 10 years in 46, XY gonadal dysgenesis reinforcing the importance of an early gonadectomy." @default.
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- W1977976472 date "1988-10-01" @default.
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- W1977976472 title "149 CERM CELL NEOPLASMS IN TWO PATIENTS WITH 46,XY KARYO-TYPE AND INTERSEXUAL CONDITION" @default.
- W1977976472 doi "https://doi.org/10.1203/00006450-198810000-00170" @default.
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