A female patient was firstly evaluated at the age of 12 years, complaining of headaches and visual loss. Physical examination demonstrated adequate height and weight, Tanner stage P1B1. Papillary edema was confirmed by fundus examination. MRI showed a pituitary macroadenoma, 6.6×7.3×6.1 cm with compression of the optic chiasm and bilateral cavernous sinus invasion. The first prolactin value obtained was 169.164 uUI/ml (normal<210) with the other pituitary axis within normal ranges for gender and age. Due to a sudden bitemporal hemianopsia and threat of vision loss, surgical debulking via classic approach was performed, and tumor volume was reduced. The histopathological examination revealed a prolactinoma with a high proliferative activity (Ki Index=17%). Subsequently, she was treated with cabergoline, with doses ranging from 1 mg to 2.5 mg/week. However, the lowest prolactin level achieved was 538,9 ng/ml (normal 30144). Tumor size decreased by only a further of 35% after two years of medical treatment. At the age of 14 year-old, she was addressed to our department for a close monitoring. After 6 months of cabergoline at a dose of 3.5 mg/week, the prolactin nadir was 150 ng/ml, with persistent gonadotropin and thyrotropin insufficiency. Her height was 154 cm (−1,4 DS) associated with an inadequate weight (+3,2 DS) and insulin resistance signs. No spontaneous signs of puberty were observed. The latest MRI revealed a persistent sellar and suprasellar mass (48/30/33 mm) with discreet extension to the cavernous sinus, but with no general mass effect. Partial optic atrophy at the ophthalmological follow-up was highlight.
Case particularities: Being this fairly rare tumor, there is a lack of treatment guidelines. The postoperative management of this case proves to be provoking, both in the control of the secretion of this large sellar mass, but as well in the ensuring of growth and puberty installation.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology