ESPE Abstracts

Ovarian steroid cell tumors (OSCT) are rare sex cord stromal tumors of the ovary and comprise <0.1% of all ovarian tumors. We herein report a case of an OSCT in a 9 year old girl patient who presented with central puberty preocious unresponsive to gonadotrophine relasing hormon analog (GnRH) therapy. A 9 year old girl presented with breast development noticed 4 months ago and menarche 3 months ago. Her height was 141,5 cm (+1,3 SDS), body weight was 35.4 kg (+0.9 SDS) and body mass index was 17.6 kg/m² (+0.5 SDS). Her thelarche was Tanner stage 3, and pubarche was Tanner stage 2. She had dysmorphic face with frontal bossing and flat nasal bridge. She also had hypertrichosis and acnes. Laboratory results were as follows: follicle-stimulating hormone (FSH): 12.2 U/l, luteinizing hormone (LH): 8.1 U/l and estradiol: 93 ng/l. On admission pelvic ultrasonographic (USG) examination uterus was 56×20 mm, right ovary 34×16 mm, left ovary 35x16 mm, and a few millimetric follicle cysts were observed. Bone age was 12 years. Cranial magnetic resonans imaging (MRI) was normal. GnRH therapy (leuprolide acetate therapy 3.75 mg/28 days) was initiated due to centrally rapidly progressing puberty and early menarche. On her control examination 3 months later, growth rate was 6.7 cm/year, breast development Tanner stage 3, and hyperpigmentation of the nipples were detected. LH and estradiol were 4.4 U/l and 420 ng/dl (very high) respectively. She did not show any clinical improvement in the third month of treatment. Her pelvic USG repeated because of increase in estradiol; uterus was 65×36×24 mm, endometrium double foldable 10 mm, fundus/cervix ratio 1.5. Right ovary was 34×28×17 mm (8.5 ml) left ovary was 15×12×10 mm (0.9 ml). A solid mass 26×20×14 mm defined as homogeneous and hyperechogenic according to ovarian parenchyma, and hypervascular. The mass in the ovary was removed laparoscopically using the ovarian-sparing surgery technique. The histopathological examination revealed benign OSCT-not otherwise specified (NOS) composed of cells positive for inhibin, Melan A, GATA3, CD10, CD31, CD56 and negative for estrogen receptor, cytokeratin, P53, desmin, S 100, and placental alkaline phosphatase. In conclusion, In this case peripheral puberty due to OSCT was combined with central rapidly progressing puberty. Estrogen-secreting ovarian masses may not be detected by USG when they are still small in size. Radiological imaging may be repeated, especially in rapidly progressing puberty cases showing clinical and hormonal progression despite treatment.