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KMID : 0357920070410010059
Korean Journal of Pathology
2007 Volume.41 No. 1 p.59 ~ p.62
A Sertoli-Leydig Cell Tumor in a Patient with Complete Androgen Insensitivity Syndrome - A Case Report -
Choi Min-Sung

Kim Dong-Won
Lee Dong-Wha
Park Sang-Mo
Jin So-Young
Abstract
Complete androgen insensitivity syndrome (CAIS) is a rare disorder sometimes referred to as male pseudohermaphroditism. This syndrome is characterized by a 46,XY karyotype with internal bilateral testes, female external genitalia, absence or scantiness of pubic and axillary hair and normal breast development. A variety of gonadal tumors can develop in individuals with CAIS. A 57-year-old patient was admitted to urology with intermittent gross hematuria for one month. CT revealed a left ureteric mass, measuring 2.5¡¿2 cm. Ureteroscopic biopsy was performed and the result was a diagnosis of high grade urothelial carcinoma. The patient underwent laparotomy which revealed slightly enlarged gonads in the pelvic cavity. However, a uterus and fallopian tubes were not identified. Left nephroureterectomy and bilateral gonadectomy were performed. The right gonad measured 3.9¡¿2.5¡¿2 cm and the cut surface showed a well circumscribed, firm, brownish gray colored 2¡¿1.8 cm sized nodule. Microscopically, this nodule consisted of uniform and solid tubules containing immature Sertoli cells and Leydig cells which were both positive for alpha-inhibin. The left gonad measured 3.5¡¿2¡¿1.9 cm and showed atrophic testicular tissue.
KEYWORD
Androgen insensitivity syndrome, Sertoli-Leydig cell tumor, Transitional cell carcinoma, Urothelial carcinoma
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