ESPE Abstracts (2016) 86 P-P2-391

Sertoli-Leydig Cell Tumor as a Rare Cause of Hirsutism in a Young Adolescent

Saskia van der Straaten, Mireille Merckx, Jo Van Dorpe, Inge Goethals, Victoria Bordon, Philippe Timmers & Jean De Schepper

UZ Ghent, Ghent, Belgium

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.

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