ESPE Abstracts (2021) 94 P2-416

ESPE2021 ePoster Category 2 Sex differentiation, gonads and gynaecology or sex endocrinology (52 abstracts)

Ovarian Juvenile Granulosa Cell Tumor in 9-Month-Old female Infant presented with Isosexual Pseudo Puberty: Case Report

Shayma Ahmed , Ashraf Soliman , Nada Alaaraj , Fawzia Alyafie , Maryam AL Maadheed & Colin Clelland

1Hamad General Hospital, Doha, Qatar; 2Sidra Medicine, Doha, Qatar

Background: In infants, less than 1-year JGCT is extremely rare, with very few reported cases in the literature.

Case Report: This 9-month old girl, presented with a day history of bloody vaginal secretions, with an increase in breast size bilaterally 2 months before presentation and growth of fine hair in the genital area. She had no skin hyperpigmentation, no skeletal abnormalities, and no dysmorphic features. On examination, the girl had no dysmorphic features. Her length = 75 cm (1.66 SD), weight= 10.4 kg (1.85SD), BMI = 18.07 kg/m2 (0.92SD). Breasts (Tanner stage 2), with fine pubic hair. Her Initial laboratory investigations showed Androstenedione 6.8 nmol/l (n=0.0-3.0), Dihydroandrostenidione sulfate 0.320 umol/l (n=0.090-3.35), estradiol 1091 pmol/l(0.0-132), FSH< <0.3 IU/l (n=1.2-12.5). LH <0.3 IU/l (n=0.3-2.5), Anti-Mullerian Hormone (AMH) >1714.0 pmol/l (n=0.1-38.6), Testosterone Level 2.2 nmol/l (n=0.0-2.2), Beta human Chorionic Gonadotropin 0.9 IU/l (n=0.0-5.0), Alpha-Fetoprotein 6 kIU/l (n=1-33) Serum Inhibin A 385 pg/ml (n<4.7), and Serum Inhibin B 3805 pg/ml (n <111). Ultrasonography showed a well-defined round-shaped heterogeneous hypoechoic lesion 62.8 ml in volume, 5.4×4.12×5.39 cm in dimension. No calcifications. Uterus developed 5.2×2.4×2.3 cm in diameter. Bone age was 1.8 years (+4.3 SDS for age and sex). MRI of the abdomen and pelvis was revealed a large solid lesion in the lower abdomen and pelvis related to the left ovary with significant gadolinium enhancement and minimal central necrosis. The uterus was enlarged. Findings were suggestive of hormone-producing sex cord-stromal tumor. Computed Tomography for the chest was normal. Based on findings above total left salpingo-oophorectomy was done through an open abdominal incision, Smooth-surfaced ovoid encapsulated mass weighing 75 g and measuring 100 × 50 × 40 mm with fallopian tube 50 mm long, attached. Solid yellow, nodular cut surfaces with no necrosis, hemorrhage, or cystic spaces. There is a small surface nodule near the Fallopian tube, 5mm across. Otherwise, the capsule is intact. The patient had no reported postoperative complications, no chemotherapy or radiation was administered. The patient sent home in the third postoperative day in good condition. The histopathology revealed GCT, Juvenile type stage pT1a, Immunohistology showed positive staining for inhibin A. At 1 month follow up, no new concerns , repeated Hormonal test showed dropping of estradiol to 62 pmol/l (0.0-132), and (AMH) to 9 pmol/l (n=0.1-38.6).

Conclusion: These 9 months-old infants with JGCT presented with peripheral precocious puberty and advanced skeletal maturation. Surgical removal was curative with regression of all hormones to prepubertal levels.

Volume 94

59th Annual ESPE (ESPE 2021 Online)

22 Sep 2021 - 26 Sep 2021

European Society for Paediatric Endocrinology 

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