Journal of pediatric and adolescent gynecology · 2013
Complete androgen insensitivity syndrome with a Sertoli-Leydig cell tumor.
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Fagouri Houda, Moussaoui Driss Rahali, Kouach Jaoud, Babahabib Abdelah, Oukabli Mohamed, Ameur Ahmed, Albouzidi Abderrahmane, Dehayni Mohamed
Аннотация
BACKGROUND: The complete androgen insensitivity (testicular feminization) syndrome was described in phenotypic females with 46XY karyotype, presenting with primary amenorrhea, adequate breast development, and absent or sparse pubic or axillary hair. Gonads consist usually of seminiferous tubules without spermatogenesis.
CASE: We report the case of a 15-year-old girl with testicular feminization since age 4. She was admitted to surgically remove the testes from the inguinal canal. Microscopic examination of the left testis revealed a Sertoli-Leydig cell tumor and the draft of fallopian tube with adjacent seminiferous tubules.
SUMMARY AND CONCLUSION: These patients have a 5%-10% risk of developing germ cell tumors. Sertoli-Leydig cell tumors are uncommon neoplasms, usually benign in testicular feminization syndrome.
CASE: We report the case of a 15-year-old girl with testicular feminization since age 4. She was admitted to surgically remove the testes from the inguinal canal. Microscopic examination of the left testis revealed a Sertoli-Leydig cell tumor and the draft of fallopian tube with adjacent seminiferous tubules.
SUMMARY AND CONCLUSION: These patients have a 5%-10% risk of developing germ cell tumors. Sertoli-Leydig cell tumors are uncommon neoplasms, usually benign in testicular feminization syndrome.