The overall prognosis for TRB is poor even with aggressive treatment, as patients usually die of disseminated neuraxis disease within the first year after diagnosis. The average survival time after diagnosis of trilateral retinoblastoma is 6–11 months, regardless of the location of the intracranial tumor [4, 6]. With aggressive multimodal treatment approach (chemotherapy, surgery, radiation), a small minority of patients can be cured. One series found that treatment appears to prolong survival from 1.3 to 9.7 months [9]. The longest reported survival after diagnosis for a TRB patient is 96 months [4]. There appears to be no difference in survival time between patients with tumors in the pineal region versus the sella region [3]. Although the location of the intracranial tumor does not affect survival, tumor size greater than 15 mm appears to be a critical size for tumor dissemination [5].

The mainstay of therapy for TRB is intensive cisplatin-based therapy (with other agents) and autologous stem cell rescue. Aggressive chemotherapy used to be followed by craniospinal irradiation (e.g., 36 Gy with boost to pineal gland to 59 Gy) in many TRB patients. Spinal metastases are very common in TRB, being present in 69–89 % of cases at autopsy [4]. However, there are serious long-term toxicities of craniospinal radiation in the very young child. Therefore, current strategies are directed toward avoiding irradiation and using intensive chemotherapy followed by autologous stem cell rescue. Surgical resection may play a role in certain cases if the intracranial disease is not disseminated. Finally, ventriculoperitoneal shunting should be avoided in TRB patients to avoid tumor dissemination into other body cavities [9].