A Sertoli-Leydig Cell Tumor in a Patient with Complete Androgen Insensitivity Syndrome: A Case Report.
- Author:
Min Sung CHOI
1
;
Dong Won KIM
;
So Young JIN
;
Sang Mo PARK
;
Dong Wha LEE
Author Information
1. Department of Pathology, Soonchunhyang University Hospital, Seoul 143-730, Korea. budapest@hosp.sch.ac.kr
- Publication Type:Case Report
- Keywords:
Androgen insensitivity syndrome;
Sertoli-Leydig cell tumor;
Transitional cell carcinoma;
Urothelial carcinoma
- MeSH:
46, XY Disorders of Sex Development;
Androgen-Insensitivity Syndrome*;
Biopsy;
Breast;
Carcinoma, Transitional Cell;
Diagnosis;
Fallopian Tubes;
Female;
Genitalia;
Gonads;
Hair;
Hematuria;
Humans;
Karyotype;
Laparotomy;
Leydig Cells;
Male;
Middle Aged;
Sertoli Cells;
Sertoli-Leydig Cell Tumor*;
Testis;
Ureter;
Urology;
Uterus
- From:Korean Journal of Pathology
2007;41(1):59-62
- CountryRepublic of Korea
- Language:Korean
-
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 x 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 x 2.5 x 2 cm and the cut surface showed a well circumscribed, firm, brownish gray colored 2 x 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 x 2 x 1.9 cm and showed atrophic testicular tissue.
