Clinical outcomes and patterns of failure in pineoblastoma: a 30-year, single-institution retrospective review.

OBJECTIVE

To update outcomes and assess prognostic factors in the modern, multimodality treatment of patients with pineoblastoma.

METHODS

The medical records of patients with pineoblastoma evaluated at the M.D. Anderson Cancer Center between 1982 and 2012 were reviewed retrospectively.

RESULTS

Thirty-one patients with medical records suitable for review were identified. The majority of patients were female (67.7%) with a median age at diagnosis of 18.2 years (range, 0.3-52.8 years). Twenty-one patients underwent surgical resection, recorded as gross total (n = 9) or subtotal (n = 12) resections. Thirty patients received radiation with photon-based therapy (n = 16), proton-based therapy (n = 13), or radiosurgery (n = 1) to a median craniospinal irradiation dose of 36 Gy (range, 23.4-40 Gy) and a median focal dose of 54 Gy (range, 40-58.4 Gy). Twenty-eight patients received chemotherapy before (n = 10), during (n = 10), and after (n = 22) radiation. Median overall survival was 8.7 years for the entire cohort, with 2-, 5-, and 10- year actuarial rates of 89.5%, 69.4%, and 48.6%, respectively. Median disease-free survival was 10 years with 2-, 5-, and 10- year actuarial rates of 84.3%, 62.6%, and 55.7%, respectively. Univariate analysis failed to correlate age, sex, or extent of surgical resection with disease-free or overall survival.

CONCLUSIONS

Modern, multimodality treatment of pineoblastoma yields a high rate of overall survival, with acceptable short- and long-term toxicity. A greater M-stage at presentation and development of disease recurrence correlate with worse overall survival. Patients who received focal radiation initially experienced a greater rate of disease recurrence compared with those treated to the craniospinal axis.