Pineocytomas

Six cases of histologically proven pineocytoma are reported. The diagnosis was established by surgery in five cases and at autopsy in one. All patients received focal radiation therapy (4500 to 5400 cGy). Two patients had local recurrences at 1 and 48 months and subsequently died of tumor. A third patient died of Alzheimer's disease 29 years after the initial presentation. Three patients are alive with no evidence of disease at 21+, 52+, and 84+ months after treatment. Tumor dissemination occurred after a local recurrence in one patient. We found no evidence of recurrence outside the irradiated field unless there was an initial recurrence at the primary tumor site. We recommend postoperative staging for all patients with pineocytoma and focal radiation therapy if local disease alone is found. Craniospinal radiation therapy appears to be justified only if tumor dissemination is documented on staging tests.