Precocious Pseudo-puberty in a Two-year-old Girl, Presenting with Bilateral Ovarian Enlargement and Progressing to Unilateral Juvenile Granulosa Cell Tumour

Ovarian causes of precocious pseudo-puberty (PPP) include McCune-Albright syndrome (MAS) and juvenile granulosa cell tumour (JGCT). We describe a case of PPP in which bilateral ovarian enlargement with multiple cysts progressed to unilateral JGCT. A girl aged 2.17 years presented with three months of breast development, and rapid growth. Examination showed tall stature, height +2.6 standard deviations, Tanner stage B3P2A1. A single café au lait patch was noted. Bone age was advanced at 5 years. Pelvic ultrasound showed bilaterally enlarged ovaries (estimated volumes 76 mL on the left, 139 mL on the right), each containing multiple cysts. Luteinizing hormone (LH) and follicle stimulating hormone (FSH) values before/after gonadotrophin administration were 0.43/0.18 and <0.1/<0.1 mUI/mL, serum estradiol 130 pg/mL, (prepubertal range <20 pg/mL). PPP of ovarian origin was diagnosed, and tamoxifen 20 mg daily started. However, after only seven weeks height velocity escalated and breast development increased to B3-4 with menorrhagia. Basal/stimulated LH and FSH were still suppressed at 0.13/0.25 and <0.1/<0.1 mUI/mL and, serum estradiol 184 pg/mL. Repeat imaging now showed normal right ovary (volume 1.8 mL) and a large left-sided vascular solid/cystic ovarian tumour which was excised (weight 850 g). Histology showed JGCT, International Federation of Gynecology and Obstetrics stage IA. DNA from tumour tissue showed no mutation in GNAS, exon 3 of AKT1 (which contains a mutational hotspot) or FOXL2. The observation that bilateral ovarian activity progressed to unilateral development of JGCT in this patient is novel. This case highlights current uncertainties in the ontology of JGCT, and its possible relationship with MAS.

Prevalence and risk factors of overweight and obesity in elementary schoolchildren in the metropolitan region of Tunis, Tunisia.

BACKGROUND: Local data about prevalence of obesity in emerging countries are rather scarce. Risk factors for obesity, well known in most industrialized countries, are poorly understood in Tunisia. AIMS: To assess prevalence of overweight and obesity and to investigate associations with possible risk factors in a group of 6-12 year- old schoolchildren in Tunis, Tunisia. METHODS: A descriptive transversal study including a sample of 1335 schoolchildren (6-12 years; mean: 9.7 ± 1.5 years) was conducted in Tunis. Personal and parental data were collected by questionnaires completed by parents. Height and weight were measured and body mass index was calculated. Prevalence of overweight and obesity was defined based on international agreed cut-off points. RESULTS: Prevalence of overweight and obesity was 19.7% and 5.7%, respectively. Risk factors associated with overweight were: high degree- educated mother and father: 17.3% vs 11.7% (p=.01) (OR (95%CI): 1.58; 1.09-2.29) and 26% vs 17.4% (p=.002) (OR: 1.66; 1.21-2.29), respectively; mother, father high in occupational hierarchy: 7.2% vs 3.6% (p=0.009) (OR: 2.1; 1.2-3.7) and 14% vs 9% (p=.014) (OR: 1.6; 1.1-2.48), respectively. Overweight children had a significantly higher consumption of bread (p=.044), of snack intake (p=0.046) and of soft drink consumption (p=.035). CONCLUSIONS: Prevalence of overweight and obesity in this cohort are 19.7% and 5.7%, respectively. Substantial differences in food choices in families with the highest socio-economic status are among risk factors contributing to obesity development.