Endocrine Abstracts

JOINT2584

Objective: Sex cord tumor with annular tubules (SCTAT) is a rare type of sex cord stromal ovarian tumor. We report an 8-year-old female who presented with peripheral precocious puberty (PPP) evolving to central precocious puberty (CPP) after excision of right ovarian tumor due to SCTAT.

Method: Single case report.

Results: She was seen in the endocrine clinic at 6 years and 8 months. She developed thelarche at 5 years and 2 months, pubic hair at 6 years, and vaginal bleeding at 6 years and 7 months. Mid parental target height is 152.3 cm. Pertinent physical examination showed height 134.2 cm (99%), weight 32.7 kgs (98%), Skin: small 0.5 cm irregular café au lait macule on the right buttock, and GU: Tanner IV breast development, Tanner 3 pubic hair. Endocrine evaluation showed LH <0.005 mIU/ml, FSH < 0.3 mIU/ml, estradiol level 121.7 pg/ml, DHEA-S sulfate 37 mg/dl, Anti-Mullerian Hormone 34.3 ng/ml (normal 0.256-6.34), β-HCG < 2 Miu/ml, TSH 0.63 uU/ml, Free T4 1.16 ng/dl, inhibin B 1,250 pg/ml (normal <182 pg/ml). Bone age X-ray of the left hand was 10 years. Pelvic ultrasonography showed uterus 6.8 × 2.7 × 4 cm and endometrium 12 mm in thickness. The right ovary measured 6.2 × 3.3 × 3.5 cm with a 2 × 4.3 × 6 cm right ovarian cyst. The left ovary measured 2.6 × 1.4 × 1.7 cm. Bone scan was negative for fibrous dysplasia. CT of the abdomen and pelvis showed right ovarian complex cystic mass. CT of the chest was negative for metastasis. She underwent right oophorectomy at 6 years 11 months. Pathology was consistent with right ovarian SCTAT with focal areas with Sertoli cells and juvenile granulosa- like appearance with classification of FIGO 1A without need for adjuvant therapy. Repeat tumor markers were normal. At follow up at 7 1/2 years, she was Tanner 5 breasts and 4 pubic hair. She had CPP with serum LH 1.4 mIU/ml, FSH 6.5 mIU/ml, and estradiol level 12.7 pg/ml. Bone age X-ray of the left hand was 11 years. She was treated with a GnRH agonist.

Conclusion: Most children with SCTAT present with PPP. Our patient progressed to CPP after right oophorectomy necessitating treatment with a GnRH agonist. Close monitoring of pubertal status and hormonal evaluation after surgery is important to detect evolution of potential CPP and manage this appropriately.