The pituitary function depends on the integrity of the hypothalamo-pituitary axis and the functionality of numerous differentiated cell lines in the anterior pituitary lobe that specialize in specific hormone production. The development of these cell lines is the result of events during pituitary organogenesis that are under the sequential control of transcription factors (1). Any abnormality occurring in the pituitary gland either congenital (congenital malformations, genetic abnormalities) or acquired (perinatal insults, tumors, infections) will cause profound alterations of the whole endocrine system.

Tumors in the pituitary region can be classified on the basis of topographic criteria as intra-, supra- para- or retrosellar. Intrasellar tumors are mostly represented by pituitary adenomas (more than 90% of all intrasellar lesions), while dysembryogenetic lesions such as Rathke’s pouch cyst are less frequent. The suprasellar tumors are dysembryogenetic lesions of the midline such as craniopharyngiomas, germinomas, dermoid or epidermoid cysts, lipomas, teratomas, hamartomas. Other tumors such as meningiomas or gliomas are uncommon during childhood or adolescence. Craniopharyngiomas, the most common cause of hypopituitarism in childhood, and adenomas are the most frequent lesions of the pituitary region in children and adolescents. Virtually all tumors of this region are benign.

This chapter aims at reviewing the most recent epidemiological, diagnostic and therapeutic knowledge on pituitary tumors in childhood and adolescence.