Endocrine Abstracts

Low-grade gliomas are the most common brain tumor in children, accounting for 30–50% of central nervous system tumors in the pediatric age group. They can occur anywhere within the central nervous system (CNS) including the optic pathway (5%). When optic gliomas involve the hypothalamus, patients will often present endocrinopathy. Signs of such involvement can include growth failure and precocious puberty. In this report, we present the case of 8 years three months girls with the history of Low-grade glioma of the Optic pathway diagnosed at the age of 1 year 8/12 in the context of visual acuity loss, nystagmus, and optic nerve pallor on examination. She underwent chemotherapy with periodic control by the carcinologiste. In the follow up since the age of 7 years she developed a progression of her glioma with concomitant linear growth acceleration. At the age of 8 years, she was referred to our department for breast development. On physical examination, his weight was 54 Kg (60–75p) and height was 141 cm (+3DS). Breast development were compatible with tanner stage 3 and no pubic hair was present. Ultrasound imaging showed ovaries were prominent (right ovary: 2× 1 cm and left ovary: 1,8×1 cm) with absence of follicles, uterus was measuring about 4×2,5 cm Biochemical measurements revealed a basal (FSH) level of 6.28 mUI/ml, LH level of 2.63 mU/ml, and Estradiol level of 58.39 ng/l. The bone age was 15 years. The patient was diagnosed with central precocious puberty and started on gonadotropin-releasing hormone (GnRH) agonist treatment (triptorelin acetate 11.5 mg every 3 months). During follow up, 6 months of starting treatment, his progression of puberty has been arrested. With this case report, we would like to emphasize that the growth acceleration in a young child presenting a glioma of the optic pathway should alert the physician to consider a diagnosis of precocious puberty at the early stage.