A Sertoli-Leydig Cell Tumor in a Patient with Complete Androgen Insensitivity Syndrome - A Case Report -
최민성, 김동원, 이동화, 박상모, 진소영,
소속 상세정보
최민성 ( Choi Min-Sung )
순천향대학교병원 병리과
김동원 ( Kim Dong-Won )
순천향대학교병원 병리과
이동화 ( Lee Dong-Wha )
순천향대학교병원 병리과
박상모 ( Park Sang-Mo )
순천향대학교병원 병리과
진소영 ( Jin So-Young )
순천향대학교병원 병리과
KMID : 0357920070410010059
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.
키워드
Androgen insensitivity syndrome; Sertoli-Leydig cell tumor; Transitional cell carcinoma; Urothelial carcinoma
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