Neonatal Cushing Syndrome: A Rare but Potentially Devastating Disease.

Neonatal Cushing syndrome (CS) is most commonly caused by exogenous administration of glucocorticoids and rarely by endogenous hypercortisolemia. CS owing to adrenal lesions is the most common cause of endogenous CS in neonates and infants, and adrenocortical tumors (ACTs) represent most cases. Many ACTs develop in the context of a TP53 gene mutation, which causes Li-Fraumeni syndrome. More rarely, neonatal CS presents as part of other syndromes such as McCune-Albright syndrome or Beckwith-Wiedemann syndrome. Management usually includes resection of the primary tumor with or without additional medical treatment, but manifestations may persist after resolution of hypercortisolemia.

Congenital adrenocortical adenoma: case report and review of literature.

Congenital ardrenocortical neoplasms are exceedingly rare. Our review of the medical literature revealed 23 reported cases of adrenocortical neoplasm including this one. Eighteen of these cases were adrenocortical carcinoma and four were grouped as adrenocortical tumor. We have not found any reported case with a histological diagnosis of a congenital adrenocortical adenoma. We present this case of a congenital adrenocortical neoplasm with histological findings consistent with an adrenocortical adenoma in a premature infant aged 27 weeks and 4 days who had a prenatal sonogram showing a cystic right abdominal mass and a physical examination demonstrating a palpable mass.