ESPE Abstracts

Background: McCune-Albright Syndrome(MAS）includes fibrous dysplasia(FD), cafe-au-lait spots and gonadotropin-independent precocious puberty(PP). Adenohypophyseal hyperfunction syndromes is uncommon but may be potentially severe complication.

Objective: To report a girl who has been confirmed McCune-Albright syndrome and has been found pituitary adenoma during follow-up.

Methods: A girl with typical type of MAS had gradually progressive manifestations due to GH/PRL secreting pituitary macroadenoma during follow-up. The clinical characteristics and managements were reviewed.

Results: The first visiting age was 4.5 years of age, with a 3 years history of recurrent breast development and virginal bleeding. She was diagnosed tipical type of McCune-Albright syndrome according to fibrous dysplasia(FD), cafe-au-lait spots and gonadotropin-independent precocious puberty accompany with recurrent ovarian cyst. At the age of first visiting, serum PRL has been found increased but no lactation, and GH as well as IGF-1 were within normal range. Letrozole was prescribed and followed by remission of the vaginal bleeding and breast development. However, she presented progressive facial and extremities asymmetric, and lactation at the age of 14 years. She also had bone fracture for twice. Her height was 173.2 cm, Body weight 61 kg. Fasting GH level 14.77ug/l, but Serum IGF-1:621ng/ml(566.9±168.9ng/ml). GH can not be suppressed by oral glucose test. No visual impairment. Thyroid function and adrenal function were normal. Macro-adenoma was confirmed by MRI scan. She accepted Bromocriptine combined with Octreotide therapy, but the PRL and GH levels remained elevated, and thus the transsphenoidal pituitary tumor resection was performed. Histological result showed that the adenoma was pituitary mammosomatotroph tumor. However, the adinoma can just be partial removed because of difficulties of the operation, due to the FD in sphenoid bone. GH levels remained abnormal after operation and octreotide therapy still needed.

Conclusion: GH/PRL secreting pituitary macroadenoma can be found in children with MAS, and can lead to severe complications. The history of the pituitary adenomas may be long and the initial manifestations may be occult. Thus long term follow up is recommended. In children with MAS and functional adenomas, Bromocriptine and Octreotide may be effective, but in some cases, other therapy including transsphenoidal pituitary tumor resection may be needed.