Clinical management of teratoma, a rare hypothalamic-pituitary neoplasia

Intracranial teratomas are rare and comprise about 0.5 % of all intracranial\r\ntumours. Actually, a total of 15 cases of sellar-suprasellar teratoma have been\r\ndescribed in the last 24 years. Although rare, hypothalamic-pituitary teratomas\r\nshould be taken into account in the differential diagnosis of\r\nhypothalamic-pituitary region tumours. The current review focuses on the clinical\r\nand therapeutic management of pituitary region teratomas. Teratomas occur more\r\nfrequently in children and young adults than in the older population and in males\r\nas compared to females. Symptoms at diagnosis are usually neurological defects,\r\ndiabetes insipidus and hypopituitarism. Teratoma diagnosis can be suggested\r\nthough neuroimaging findings. Magnetic resonance imaging remains the preferred\r\nmodality for assessment of teratoma. Neuro-radiological findings of mixed-density\r\nmass, usually with mixed cystic and solid components or inclusions of teeth, fat \r\nand calcification can be suggestive. Tumour markers as beta-HCG and alpha-FP can \r\nbe useful at teratoma diagnosis for distinguishing immature teratomas, mixed GCTs\r\nand mature teratomas with immature or malignant components. Optimal treatment for\r\nmature teratoma is neurosurgical excision. Radical excision is advocated as\r\nrecurrence rate for a mature teratoma is extremely low in cases of complete\r\nresection and long-term outcome is excellent. During post-treatment follow-up, in\r\ncases of healing, according to tumour marker evaluation and contrasted MRI\r\nfindings, hormone replacement therapy should be considered, also for secondary\r\nhypogonadism and GH deficit, with a more intense follow-up. However, as actually \r\nfew evidence are available, safety data have to be confirmed also trough a\r\nsurveillance study.