Trilateral retinoblastoma

Trilateral retinoblastoma is a relatively uncommon condition seen in around 5% to 15% of patients with hereditary retinoblastoma (1,2).

• it is a primary pineal tumour or an ectopic intracranial tumour which develops in the pineal or suprasellar region in combination with bilateral retinoblastoma (1,3)
• does not result from metastasis of intraocular retinoblastoma

The condition is usually identified 2 years after the diagnosis of intraocular retinoblastoma (but may occur up to 11 years of age) (2). Patients may present with headache, vomiting, hydrocephalus, and meningismus (3)

Median survival time from diagnosis of the disease has been estimated to be 9 months (2).

• asymptomatic patients at the time of diagnosis have a better outcome than patients who presents with symptomatic trilateral retinoblastoma (2)
• routine neuroimaging using magnetic resonance imaging (MRI) in patients with bilateral disease has been recommended (1)

Reference:

• (1) National Cancer Institute 2012. Retinoblastoma treatment
• (2) McDaid C et al. Systematic review of effectiveness of different treatments for childhood retinoblastoma. Health Technol Assess. 2005;9(48):iii, ix-x, 1-145.
• (3) Melamud A, Palekar R, Singh A. Retinoblastoma. Am Fam Physician. 2006;73(6):1039-44.