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
The Klinefelter testis is morpholOgically normal at birth in most cases. Primary spermatogonia are already greatly reduced in number by late childhood. Shortly before the expected time of puberty, the seminiferous tubules begin to degenerate. The absence of elastic fibers in the tubular wall indicates that the process of atrophy began prepubertally. The testes in adult 47 XXY individuals are small and rarely exceed 2 cm in maximal dimension (Fig. 19). On microscopic examination, they are largely atrophiC, have hyalinized seminiferous tubules, and a relative increase in the number of Leydig cells.
23); similar changes occur in the streak gonads of Turner syndrome (45 X karyotype). Approximately one-third of patients with MGD develop gonadoblastoma, a tumor found almost exclusively in patients with an intersex syndrome and a Y chromosome. 40,50,51 Gonadoblastoma accounts for three-fourths of the gonadal tumors arising in dysgenetic gonads and is usually discovered during the first to fourth decades oflife. Many of the isolated reports of gonadoblastoma associated with other forms of hermaphroditism described clinically and patholOgically actually may be examples ofMGD.
Science 255: 453--456 Hudson PL, Dougas I, Donahoe PK, Cate RL, Epstein J, Pepinsky RB, MacLaughlin DT (1990) An immunoassay to detect human miillerian inhibiting substance in males and females during normal development. J Clin Endocrinol Metab 70: 16--22 Imperato-McGinley J, Miller M, Wilson JD, Peterson RE, Schackleton C, and Gajdusek DC (1991) A cluster of male pseudohermaphrodites with 5 alpha-reductase deficiency in Papua New Guinea. Clin Endocrinol34: 293--298 Jager RJ, Epensperger C, Fraccaro M, Scherer G (1990) A ZFY negative 46,XX true hermaphrodite is positive for the Y pseudoautosomal boundary.
Blaustein’s Pathology of the Female Genital Tract by Stanley J. Robboy M.D., Peter F. Bernhardt M.D., Tim Parmley M.D. (auth.), Robert J. Kurman M.D. (eds.)