Background: Sertoli-Leydig Cell Tumors (SLCT) account only for 1% of all ovarian neoplasia, occur more commonly in the second or third decade of life and seldom secrete tumor markers. The experience in adolescence is limited.
Objective and hypothesis: To report the hormonal and biological profile of a SCLT in a young adolescent. Ovarian tumor markers as well as FDG-PET scanning might be helpful in diagnosing ovarian malignancy in case of normal ultrasound imaging.
Results: Mildly elevated androsteendion (4.35 ng/ml) and 17 hydroxy progesteron (4.6 ng/ml), but a markedly elevated testosterone (425 ng/dl), but normal cortisol (12 μg/dl), SHBG (27 nmol/l)), LH (7 U/l), FSH (4.9 U/l) and estradiol (40 ng/ml) were measured in a 13 years old girl (A3P6M5) with increasing hirsutism, slight acne, voice deepening and menses irregularity, but without male pattern baldness or cliteromegaly at examination. ACTH testing showed a normal cortisol and androgen response. Ultrasound of the adrenals and ovaries were normal. Hormonal re-analysis confirmed the elevated testosterone concentration by LC MSMS (222 ng/dl), but showed normal 17 OH progesterone, DHEAS and androstenedion concentrations. A search for ovarian tumor markers showed an elevated AMH 17.4 mcg/l and a markedly elevated Alfa fetoprotein (268 mcg/l), but a normal inhibin B and b hCG concentrations. A FDG PET CT scan showed an oval mass with sharp borders (3.1×2.4 cm) with high peripheral uptake in the right ovary. Histopathologically and histochemically the diagnosis of SLTC was confirmed.
Conclusion: In testosterone producing SLCT, non suppressed gonadotropin levels, probably by a low degree of aromatization to estradiol, as well as elevated alfa fetoprotein concentrations can be observed. FDG-PET scanning might be helpful in diagnosing ovarian malignancy when ultrasound imaging is normal.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology