A Case of Teratocarcinoma with Central Diabetes Insipidus / 소아과

Central diabetes insipidus is a rare disorder that can result as a consequence of diverse etiologies, including malformations, autoimmune, infiltrative(e.g. neoplastic or histiocytosis) or traumatic processes, as well as mutations in the gene encoding arginine vasopressin. Idiopathic central diabetes insipidus is a diagnosis of exclusion, one that has been made less frequently through the decades. Idiopathic central diabetes insipidus in children and adolescent requires a frequent follow-up regimen using serial brain MRI and CSF examinations especially if an isolated pituitary stalk thickening or loss of a hyperintense signal in the posterior lobe is observed. Also, so-called "idiopathic" central diabetes insipidus warrants close follow-up to determine the etiology, especially if anterior pituitary hormone deficiencies are detected. We report a case of idiopathic central diabetes insipidus with growth hormone deficiency and loss of a hyperintense signal in the posterior lobe of pituitary in the brain MRI. We followed up with serial contrast enhanced brain MRI and CSF evaluation for the early detection of an evolving occult hypothalamic-stalk lesion and finally detected a newly developed teratocarcinoma in the suprasellar region.

A Case of Central Diabetes Insipidus with Growth Hormone Deficiency and Loss of Hyperintense Signal in the Posterior Lobe / 대한소아내분비학회지

Central diabetes insipidus is a rare disorder that can result as a consequence of diverse etiologies, including malformations, autoimmune, infiltrative(e.g. neoplastic or histiocytosis) or traumatic processes, as well as mutations in the gene encoding arginine vasopressin. Idiopathic central diabetes insipidus is a diagnosis of exclusion, one that has been made less frequently through the decades. Idiopathic central diabetes insipidus in children and adolescent requires a frequent follow-up regimen using serial brain MRI and CSF examinations especially if an isolated pituitary stalk thickening or loss of a hyperintense signal in the posterior lobe is observed. Also, so-called "idiopathic" central diabetes insipidus warrants close follow-up to determine the etiology, especially if anterior pituitary hormone deficiencies are detected. We report a case of idiopathic central diabetes insipidus with growh hormone deficiency and loss of a hyperintense signal in the posterior lobe. He is a 13 years old. We are scheduled to follow-up with serial contrast enhanced brain MRI and CSF evaluation for the early detection of an evolving occult hypothalamic-stalk lesion.