Korean J Pathol.  2007 Feb;41(1):59-62.

A Sertoli-Leydig Cell Tumor in a Patient with Complete Androgen Insensitivity Syndrome: A Case Report

• Choi MS
• Kim DW
• Jin SY
• Park SM
• Lee DW

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.

Keyword

Androgen insensitivity syndrome; Sertoli-Leydig cell tumor; Transitional cell carcinoma; Urothelial carcinoma

MeSH Terms

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