Background: Multiple Endocrine Neoplasia Type 1 (MEN1) is an autosomally transmitted hyperplastic or neoplastic disorders of some endocrine and non-endocrine organs. Pituiatry tumors develop in 3070% of patients with MEN1. Mean age at onset of MEN1 associated pituitary tumors is the 4th decade and its occurence before and during puberty is very rare. Although there are two case reports about MEN1 and delay puberty, early and rapidly progressive puberty with MEN1 has no reported yet.
Case: Eight years and 5 months girl whose father has MEN1, applied with pubic and axillary hair that were detected first 10 months ago. At that time, her tanner stage was 2. The patient was diagnosed as central precocious puberty as a result of LHRH test. Bone age was advanced (2.5 years more than chronological age). The predicted adult height was calculated considerably shorter than the target height and GNRH anologue treatment was started to patient. In pituitary MR, hypointense region which was stabil in size during the follow up, was seen in intermediate lob but it was not interpreted in favor of the adenoma. In biochemical tests at admission, serum calcium level was high whereas serum phosphorus, PTH and 25-OH vit D levels were normal. In follow up, PTH increased, hyperparathyroidism was detected and patient was diagnosed as MEN1 same as her father. Furthermore, growth velocity decreased and patient needed also growth hormone therapy.
Conclusion: This case emphasize relevance of early screening of endocrine disorders for members of families with MEN1 because of diversity of endocrine disorders and also it should be kept in mind that rare endocrine presentations as precocious puberty can also be detected in the follow up of patients with MEN1.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology