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Rua Dr. Alceu de Campos Rodrigues, 247 - 12Â° and. Cj. 21
SÃ£o Paulo/SP - Brasil - CEP 04544-000
Liberman B; Nogueira KC; Huayllas MC; Goldman J; Cukiert A.
Departments of Endocrinology and Neurosurgery, Hospital Brigadeiro, Sao Paulo SP, Brazil
Rationale: Rathke´s cyst and ACTH-secreting adenoma are very rare pituitary lesions. We describe a patient presenting as Cushing’s disease in whom both lesions were documented.
Case Report: RCS, a 23 years-old lady, presented with weigh gain over the last 4 years, associated to acne, hypertricosis and muscle weakness. She had a typical Cushing’s phenotype, diabetis mellitus and hypertension. Plasma cortisol and ACTH levels were 17 mg/dL (non-suppressive) and 79.6 ng/ml, respectively, and free urinary cortisol level was 452 ug/24 hours. MRI showed a hypointense posterior sella cyst both on T1 and T2 sequences, and an otherwise normal adenohypophisis. She was submitted to transesphenoidal surgery and an intrasellar cyst containing mucoid material was evacuated. Pathological examination confirmed the presence of a Rathke´s cyst. There was no modification in her hypercortisolemia. A post-operative MRI showed that the intrasellar cyst had been completely removed. The adenohypophisis remained normal-looking on MRI. She was submitted to bilateral petrosal sini catheterism and sampling which showed a central ACTH-secreting dynamics. She was submitted to a second transesphenoidal surgery, during which a microadenoma was found within the right portion of the pituitary gland. She developed adrenal insufficiency in the immediate postoperative period. Pathological examination found an ACTH-secreting adenoma.
Discussion: The association of ACTH-secreting adenoma and Rathke´s cyst is extremely rare. Although Rathke´s cysts might remain unchanged for long periods, many of them are quite big or can show increase in size during follow-up and need surgical removal. When this dual pathology is present, we believe that both lesions should be resected.