Giant pituitary macroadenoma at the age of 4 months: case report and review of the literature

Abstract

Adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma is extremely rare. Only five cases are reported in the literature to date. We report the youngest patient of Cushing's disease due to ACTH-secreting pituitary macroadenoma at the age of 4 months. The patient presented with cushingoid facial features, hypertension and optic atrophy. Serum cortisol level was 97 microg/dl, with increased secretion of urinary free cortisol; plasma ACTH level was 353 pg/ml. Magnetic resonance imaging of the brain showed a large contrast-enhancing solid mass sitting in the sellar region, with suprasellar and lateral extension. Surgical resection was done, and immunohistochemistry confirmed the diagnosis of ACTH-secreting pituitary adenoma. Postoperatively, the patient died of disseminated intravascular coagulopathy. Cushing's disease due to ACTH-secreting pituitary macroadenoma is a possible diagnosis in early infancy. This report, along with five previously reported cases in the literature, revealed a poor surgical outcome. This surgical morbidity is possibly attributed to difficult peroperative hemostasis due to vasculopathy in Cushing's disease, which leads to excessive blood loss, adverse hemodynamic changes, myocardial dysfunction and disseminated intravascular coagulopathy.