Cushing's disease in a neonate is rare and has been reported secondary to pituitary macroadenoma. A case of Cushing's syndrome due to congenital immature teratoma in the region of the pituitary has never been reported. We discuss a case of a neonate who presented with Cushing's syndrome secondary to a congenital immature teratoma in sellar, suprasellar and parasellar regions with ectopic ACTH secretion, thereby mimicking Cushing's disease. The management issues and prognosis of congenital teratomas and neonatal Cushing's syndrome have been discussed. We describe the first case of intracranial ectopic ACTH secreting teratoma in a young infant. The prognosis is usually bad unless total excision is achieved. In the preoperative period and in case of subtotal excision, chemotherapy to take care of hypercortisolemia may be given.
Copyright 2010 S. Karger AG, Basel.