By Stanley J. Robboy M.D., Peter F. Bernhardt M.D., Tim Parmley M.D. (auth.), Robert J. Kurman M.D. (eds.)
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Additional info for Blaustein’s Pathology of the Female Genital Tract
20. Streak ovary (arrow) in Tnrner syndrome. The streak gonad is thin, fibrous, and lacks germ cells. (From Welch and Robboy, ref. ) 21 1. Embryology of the Female Genital Tract tex, a medulla, and a hilus. The cortex is composed of characteristic ovarian stroma in which the cells are elongated, wavy, and comprise conspicuous nuclei and scant cytoplasm. Rete tubules (rete ovarii) and hilar cells are typically present in the hilus region. Oocytes are almost always absent in adults with Turner syndrome.
A second potential mechanism that might lead to the Y( -) condition is chromosomal mosaicism with a prevalent XX lineage. In such patients, the Y-containing cell line might simply be technically too difficult to identifY because of the small number of such cells. Alternatively, a 47 XXY zygote might lose its Y chromosome by nondysjunction early in ontogeny, thus allowing a 46 XX cell line to persist; the 47 XXY cell line may have persisted long enough to induce male gonadal development. Such patients, just as patients with familial Y( - ) XX maleness, often present with sexual ambiguity suggesting that patients with Y( -) XX male syndrome are closely related both phenotypically and etiolOgically to XX true hermaphrodites, who present with both testicular and ovarian tissue.
Dean HJ, Shackleton CHL, Winter JSD (1984) Diagnosis and natural history of 17-hydroxylase deficiency in a newborn male. J Clin Endocrinol Metabol59: 513--520 8. Disteche CM, Casanova M, Saal H, Friedman C, Sybert Y, Graham J, Thuline H, Page DC, Fellous M (1986) Small deletions of the short arm of the Y chromosome in 46,XY females. Proc Nat! Acad Sci 83: 7841-7844 9. Donahoe PK (1992) Mullerian inhibiting substance in reproduction and cancer. Mol Reprod Dev 32: 168-172 10. Eicher EM, Beamer WG, Washburn LL, Whitten WK (1980) A cytogenetic investigation of inherited true hermaphroditism in BALB/cWt mice.