PINEALOBLASTOMA IN ADULTS

THIS IS THE first report of a series of adults (>16 years of age) with pineoblastomas who had their entire neuraxis staged at the time of di agnosis. Between 1975 and 1992, seven men and four women with histolog ically proven pineoblastomas were evaluated at the University of Calif ornia, San Francisco. The median age at diagnosis was 36 years (range, 17-59 yr). All patients presented with symptomatic hydrocephalus. One patient had a complete surgical resection, eight had subtotal resecti ons, and two had biopsies only. One patient refused any treatment or f ollow-up review and died 6 months after diagnosis. The five patients w ith positively staged disease had progression either focally or in the spine 8 to 49 months (median, 10 mo) after initial diagnosis and died 1 to 20 months after recurrence; the median overall survival time fro m the date of surgery was 30 months. In contrast, all five patients wi th negatively staged disease were alive without disease progression af ter a median of 26 months of follow-up. Our retrospective review shows that the extent of disease at diagnosis seems to be an important prog nostic factor for pineoblastomas, as is true for medulloblastomas and other primitive neuroectodermal tumors. Initial staging should include examination of the cerebrospinal fluid and magnetic resonance imaging of the spine. Although patients with pineoblastomas are often treated with adjuvant systemic chemotherapy after craniospinal irradiation, t he benefits of this approach are unclear.