MEN-1 PRESENTING AS CUSHING’S DISEASE
Liberman B; Nogueira KC; Huayllas MC; Goldman J; Cukiert A.
Departments of Endocrinology and Neurosurgery, Hospital Brigadeiro, Sao Paulo SP, Brazil
Signs or symptoms derived from pituitary adenoma represent 25% of the initial patterns of clinical presentation in patients with MEN-1. Prolactin-secreting adenoma is by far the most common of them (up to 60% of all MEN-1 related pituitary tumors) and ACTH-secreting are extremely rare, especially as the initial clinical presentation. We report a patient with MEN-1 who initially presented as Cushing´s disease.
MRFR, a 34 years-old lady, presented with weigh gain and amenorrhea over the last year. Biochemical investigation disclosed ACTH-dependent hypercortisolemia. Plasma cortisol and ACTH levels were 21.4ug/dl (non-suppressive) and 37.7pg/ml, respectively. Free urinary cortisol level was 160 ug/24 hours. Both plasma cortisol and ACTH were responsive during a DDAVP activation test. MRI showed a 4-mm microadenoma. Low calcium (13,1 mg/dl), high phosphate (2,5 mg/dl) and parathormone (149 pg/ml) levels were noted during regular biochemical screening performed in all patients with pituitary tumor. Gastrin plasma level was 126 pg/ml. Scintilographic imaging (sestamibe) was suggestive of hyperparathyroidism. She was submitted to cervicotomy and transesphenoidal surgery. Parathormone plasma level normalized after cervicotomy and the patient developed adrenal insufficiency after transesphenoidal surgery. Pathological examination of the specimens obtained from the above mentioned procedures found parathyroid hyperplasia and ACTH-secreting pituitary adenoma.
This patient presented with Cushing’s syndrome and silent hyperparathyroidism. Cushing’s disease is a very rare pattern of clinical presentation in patients with MEN-1 (less then 2%). Up to 80% of these patients might have entero-pancreatic tumors but there was no abdominal tumor documented so far in this patient. Routine screening for MEN-1 in patients with pituitary tumor might increase our ability to further diagnose this syndrome.