ESPE Abstracts

Among the acquired causes of growth hormone deficiency (GHD) in childhood, the most common reasons are benign or semimalign pituitary tumors - first and foremost craniopharyngiomas or dysgerminomas. We report on a very rare differential diagnosis in a 11-year-old, prepubertal boy with a growth arrest (1.1 cm in 2 years, height - 2.38 SDS). 2 growth hormone stimulation tests confirmed GHD (2,7 and 2,3 ng/ml after priming). There was mild central hypothyroidism (fT4 1.04 ng/dl, T3 0.9 ng/ml) and normal morning cortisol (10.9 mcg/dl) - thyroxin was substituted and a cortisol stress dose was prescribed. Nocturia was reported, but there was no polydipsia. Cranial MRI showed a 12x8x7mm cystic pituitary lesion with the strong suspicion of craniopharyngeoma. As calcifications were absent in the lesion on MRI, dysgerminoma was excluded by lumbar puncture and a short-term control MRI was arranged. The likely consequence of surgical removal was explained to the family. 3 months later a regression in size (10x7x6mm) was documented on MRI-consequently surgery was postponed and repeated MRIs performed. Over the course of 2 years the lesion showed further regression (5x3x3mm 9 months after presentation), the protein rich (hemorrhagic) areas were resorbed. The latest MRI- 2 years after first presentation- only showed minor residual enlargement of the pituitary stalk. Retrospectively, radiologists and neuro-oncologists consider hemorrhagic apoplex of a Rathke cleft cyst (RCC) as the most likely diagnosis. Over the observation period, therapy with thyroxin was continued, GH was substituted starting 6 months after diagnosis, together with ADH which improved persistent nocturia, and puberty was induced at age 14,5 years. Summary and conclusion: Rathke cleft cysts (RCC) are benign sellar and suprasellar lesions found in as many as 1 out of 6 healthy people, they are often asymptomatic and found incidentally. In the literature there are very few case series of describing hemorrhagic apoplexy oft the RCC with consecutive pituitary insufficiency. All diagnoses had been made histologically upon surgery. In our patient we interprete the growth arrest prior to diagnosis as developing pituitary insufficiency caused by a RCC with asymptomatic hemorrhagic apoplex. Interestingly, there was significant regression over time with almost normal findings upon the latest MRI thus saving the child from pituitary surgery. The endocrine long-term outcome still hast to be evaluated after attainment of final height.