Endocrine Abstracts

Background: A 5-month-old girl presented with a history of acute abdominal distension over several weeks. Three weeks previously she had developed pubic hair, and some early breast development. The GP had reassured parents on two occasions that this was normal. On examination she had Tanner breast stage 3 bilaterally, pubic hair stage 3, mild cliteromegaly, and gross abdominal distension.

Investigations: An ultrasound scan revealed a massively enlarged right ovary measuring 10 cm×6.7 cm×7.7 cm. The left ovary appeared normal. The uterus was adult in configuration with a thickened endometrium of 8 mm. There was ascites throughout.

Bone age on TW3 scoring was not advanced.

Progress: Right salpingo-oophorectomy was performed (right ovary weighing 260 g). At operation the ovarian capsule was noted to be breached. Histology confirmed a Juvenile Granulosa Cell tumour. No tumour cells were reported in the peritoneal fluid. The tumour was staged as Stage 1c on account of there being a capsular breach and a mitotic rate of up to 23 mitoses per high power field but no evidence of distant metastases. Her baseline and post-op blood investigations are presented in Tables 1 and 2. In view of this she received postoperative chemotherapy.

Within 3 weeks tumour markers had normalised, and pubertal staging had regressed to Tanner stage 1.

Conclusion: Precocious puberty in infancy must always be investigated promptly.