ABSTRACT
Tumors arising in the pineal area are heterogeneous in nature and include the pineal parenchymal tumor, pineoblastoma, pineocytoma, germ cell tumor, and papillary tumor of the pineal region. The pineal region is defined as an area surrounded by the splenium of the corpus callosum and tela choroidea dorsally, the quadrigeminal plate and midbrain tectum ventrally, the posterior aspect of the third ventricle rostrally, and the cerebellar vermis caudally. Pineal tumors account for greater than one percent of all primary brain tumors in Europe and North America but are more common in Asian countries. The clinical signs of pineal area tumors may include raised intracranial pressure and/or focal neurological symptoms related to the presence of the pineal tumor itself. The duration of symptoms before diagnosis is related to tumor growth velocity. Pineal parenchymal tumor of intermediate differentiation is associated with a good outcome, with a median progression-free survival of >5 years and median overall survival between 13 and 15 years.
