Trilateral retinoblastoma. Pineoblastoma (ectopic intracranial retinoblastoma) associated with bilateral retinoblastoma.

A 5-month-old healthy boy had bilateral retinoblastoma. The left eye was enucleated and the right eye was successfully treated by external radiation. At the time when this treatment was completed, a large midline tumour mass in the sellar and parasellar areas of the brain was discovered by CT scan. This tumour was also treated by external radiation, but a recurrence was observed after three months. The patient died 11 months from the diagnosis of retinoblastoma. At autopsy the orbits showed no extraocular growth and the treated eye revealed only a scarred tumour. There was no evidence of metastatic disease. The brain tumour resembled pineoblastoma or differentiated retinoblastoma. It is concluded that the case shows all clinical features of 'trilateral' retinoblastoma, which could be confirmed by complete autopsy as well as neuropathological and ophthalmopathological studies.