Treatment

Spontaneous regression is seen in some patients (1).

Treatment options available for intraocular retinoblastoma include:

• enucleation
  • useful in the treatment of advanced disease where there is often no expectation that useful vision can be salvaged (2)
  • in large unilateral tumours -
    • curative in >95% of patients with unilateral disease (3)
  • in bilateral tumours -
    • enucleation is indicated for the worst affected eye and external radiation to the contralateral eye (2)
    • rarely indicated for both eyes when both have advanced disease (2)
  • the remaining eye should be monitored closely for orbital recurrence of the disease (specially during the first 2 years after enucleation) (4)
• external-beam radiation therapy (EBRT)
  • used for the treatment of bilateral retinoblastomas with active or recurrent disease after completion of chemotherapy and local therapies (3)
  • also beneficial when the tumour has extended into the orbit, or is located near the optic disc or fovea (2)
• brachytherapy
  • can be used as the primary treatment or following chaemoreduction or to treat recurrent or residual tumours that are not successfully controlled by initial therapy
• thermotherapy
  • used as primary therapy for small tumours or in combination with chaemothaerapy for large tumours (4)
• cryotherapy
  • useful for the primary treatment of small peripheral tumours or for small recurrent tumours previously treated with other treatment methods
• laser photocoagulation
  • recommended only for small posterior tumours
• chemotherapy
  • used
    • to reduce the tumour volume to make it accessible to subsequent local ophthalmological therapies (cryotherapy and laser photocoagulation, or thermotherapy) to eradicate the remaining disease
    • alone as primary treatment
    • in combination with EBRT in advanced intraocular retinoblastoma to avoid enucleation (2)
    • main treatment of choice in extraocular or trilateral retinoblastoma (2)
  • more recently, direct delivery of chemotherapy into ophthalmic artery has been used as a feasible and effective method for ocular salvage (4)

Patients with heritable retinoblastomas should be offered genetic counselling (4).

The two treatment centres in the UK for retinoblastoma are the Royal London Hospital and Birmingham Children's Hospital.

Reference:

• (1) Retinoblastoma; RB1. Online Mendelian Inheritance in Man (OMIM) 2012
• (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) Chintagumpala M et al. Retinoblastoma: review of current management. Oncologist. 2007;12(10):1237-46
• (4) National Cancer Institute 2012. Retinoblastoma treatment
• (5) Carter J. Retinoblastoma. GPonline 2009.