Papillary tumor of the pineal region

Presented is a patient with papillary tumor of the pineal region (PTPR), an uncommon and recently recognized neoplasm. As its name implies, PTPR does not arise from the pineal gland itself. The cell of origin is thought to be the specialized ependymocytes of the subcommissural organ. Primary tumors of the pineal region include pineal parenchymal neoplasms, germ cell neoplasms, and tumors arising from adjacent structures, including meningiomas, astrocytomas, and ependymomas. Like other masses in this location, PTPR often leads to obstructive hydrocephalus. Due to the relative paucity of reported cases of PTPR, its natural history is unknown.