Abstract
Gonadal histology was investigated by means of conventional microscopy in 11 Complete Adrogen insensitivity Syndrome (CAIS), in 5 Incomplete Adrogen Insensitivity Syndrome (IAIS) and in 4 5αreductase deficiency (5αR-D) cases. Testicular tissue was removed as a prophylactic measure in all patients.
Age range was 1.8 - 18.6 yrs; 12 were prepubertals and 8 pubertals.
In the CA1S group 1 patient (18.6 yrs, pubertal) showed a “carcinoma in situ” pattern of the germ cells, 3 (1.8 Yrs; 9.8 yrs, prepubertal; 4.8 yrs, pubertal) had severe dysplasia, 5 patients showed a mild or moderate dysplasia and 2 had a normal pattern. In the IAIS group, 2 (5.8 yrs; 11.25 yrs, prepubertal) showed a “carcinoma in situ” pattern, 1 (10.5 yrs, prepubertal) had a severe dysplasia. 2 showed mild dysplasia. In 5α R-D we found 1 patient (13 yrs, pubertal stage) with severe, 2 with mild and 1 with no displasia.
It should be noted that; 1) Pseudohermaphroditisms with different pathogenesis all show frequent histological alterations. The reason for these abnormalities has yet to be fully examined. However, the abnormal position of the testes is the factor common to these disorders. 2) No correlation was found between the age at gonadectomy and the gravity of cellular abnormality.
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Cassio, A., D'Errico, A., Balsamo, A. et al. 150 GONADAL HITOLOGY IN TWENTY SUBJECTS WITH MAL PSEUDO – HERNAFRODITISM. Pediatr Res 24, 542 (1988). https://doi.org/10.1203/00006450-198810000-00171
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DOI: https://doi.org/10.1203/00006450-198810000-00171