Pinealoblastoma
Pinealoblastoma is a malignant tumor of the pineal gland. A pinealoblastoma is a supratentorial midline primitive neuroectodermal tumor.
Pinealoblastoma may occur in patients with hereditary uni- or bilateral retinoblastoma. When retinoblastoma patients present with pinealoblastoma this is characterized as "trilateral retinoblastoma". Up to 5% of patients with hereditary retinoblastoma are at risk of developing trilateral retinoblastoma. Prognosis of patients with trilateral retinoblastoma is dismal, only a few patients have survived more than 5 years after diagnosis; all survivors were diagnosed with small tumors in a subclinical stage. Recent advances in chemotherapy treatment regimens and early detection have improved survival of patients with trilateral retinoblastoma to up to 50%.
