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Neuroendocrinologia | Trabalhos em Congressos

ISOLATED CUSHING’S DISEASE IN IDENTICAL TWINS

Cukiert A, Andrioli M, Goldman J, Nery M, Salgado LR, Pimentel F, Knoepfelmacher M, Liberman B, Jacomossi A.

Serviços de Neurocirurgia e Endocrinologia do Hospital Brigadeiro, São Paulo SP

Genetic and acquired ethiologies had already been discussed in the genesis of ACTH-secreting tumors. MPC, a 12-years old girl presented with weight gain and low stacture. She has a normal identical twin sister. The phenotype was that of Cushing’s syndrome. She was 1,46 m high (much lower than the normal sister). There was no visual loss. Serum cortisol was 33.6 m g/dl, ACTH was 38 pg/ml and urinary cortisol was 453 m g/ 24 hours (normal range from 20 to 90). There was no suppression with low dose dexametasone but she effectively suppressed with high doses. MRI disclosed a macroadenoma occupying the entire sella with deviation of the pituitary to the left side. The patient was operated through the transesphenoidal route and underwent clinical remission of hypercortisolism. She re-started to grow, but is still shorter then the healthy sister. Three years after treatment, her sister remains healthy. The finding of a ACTH-secreting tumor in only one of identical twins strongly suggests that acquired factors may play an important role in the development of these tumors.