The Sertoli-Leydig cell tumor is a gonadal tumor of sex-cord stromal type, similar to that seen in of the various phases of testicular development in the male. This tumor is exceedingly rare, accounting for only 0.1% to 0.5% of all primary ovarian neoplasms. It occurs predominantly in the second and third decades(mean age about 25 years), less than 10% after menopause. We investigated a case of poorly differentiated Sertoli-Leydig cell tumor of right ovary, occured in a 76-year-old woman. Grossly, the tumor measured 2, 100 gm in weight and 25 x 19 x 8 cm in dimensions. The outer surface was smooth and glistening without rupture of the capsule. Cut sections revealed a multilobulated brown solid mass with multiple cystic change. Microscopically, it showed the typical findings o a Sertoli-Leydig cell tumor.
The characteristic feature is hemangiopericytoma paftem of sarcomatoid spindle cells. Therefore, we present it with a brief review of the literature.
Sertoli-Leydig cell tumors of the ovary are rare, accounting for no more than 0.5% of all ovarian neoplasms. These tumors occur predominantly in women 10 to 30 years of age.
Heterologous elements are found in over 20% of the moderately and poorly differentiated Sertoli- Leydig cell tumors. The most common element is gastrointestinal mucin-secreting epithelium, found in approximately 20% of all cases. Approximately 4% of Sertoli-Leydig cell tumors contain immature skeletal muscle and/or cartilaginous elements. The origin of these neoplasms is regarded as neometaplasia of the mesodermal neoplastic element. While the degree of differentiation of Sertoli-Leydig cell tumors is probably of greater importance in determining prognosis than the presence of heterologous components, recent evidence does point to an unfavorable effect on clinical outcome when heterologous mesenchymal components such as cartilage and striated muscle are present. We experienced two cases of ovarian heterologous Sertoli-Leydig cell tumors with gastrointestinal type epithelium and striated skeletal muscle in 20 and 21 year-old females, respectively. One case involved a patient who had complained of amenorrhea for 3 years while the other case involved a patient who had complained of left lower abdominal tenderness and amenorrhea for one and one half years. It was determined by ultrasonography of the pelvis that the two patients suffered from right and left ovarian masses. Both patients underwent adnexectomy. These tumors were large with partly solid and partly cystic areas. Based on light microscopic examination one case was classified as an intermediate Sertoli-Leydig cell tumor with heterologous gastrointestinal type epithelium, while the other case was classified as a poorly differentiated Sertoli-Leydig cell tumor with heterologous striated skeletal muscle.
Sertoli-Leydig cell tumor or Brenner tumor is a relatively rare ovarian tumor. Other associated elements in the form of epithelial, mesenchymal, and tumor components of each tumor have been reported. The Sertoli-Leydig cell tumor with a Brenner tumor element has not been documented in the literature, so we are reporting on a case of Sertoli-Leydig cell tumor coexisting with a Brenner tumor. This 62-year-old woman presented with a 4 year history of lower abdominal mass and vaginal bleeding. Exploratory laparotomy was done.
The left ovary showed a multiseptated, cystic, yellow-white solid mass, measuring 17.0x13.0x5.0 cm and weighing 985.0 gm. Microscopic examination revealed a tubular or trabecular arrangement of the Sertoli cells and variable numbers of Leydig cells. There were several tumor nests with cystic spaces composed of ovoid cells showing longitudinal nuclear grooving. Sarcomatoid or heterologous elements were not found.
A 26-year-old woman had an ovarian Sertoli-Leydig cell tumor (SLCT) associated with an elevated level of serum alpha-fetoprotein (AFP). The tumor had a heterologous element of intestinal-type mucinous epithelium, retiform and intermediately differentiated tubules of the Sertoli cells, and AFP-producing Leydig cells. AFP was demonstrated within the Leydig cells by an immunohistochemical technique. After surgery, the serum AFP level of the patient fell to the normal range. The present case is the first documented case of AFP producing a SLCT of the ovary reported in Korea.
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.