In rare instances, infants and young children (often less than 2 years of age) develop ‘leukocoria’, or a white spot in their eye. A genetic mutation triggers a set of immature retinal cells to turn malignant in these children. The unchecked growth of this cell mass, now a tumor, leads to an eye cancer called retinoblastoma.

Most cases of retinoblastoma have a preponderance for one eye alone. Retinoblastoma is the most common eye cancer in children. However, there are very few facilities to screen and identify this cancer in children, not to mention treating them. In India and other developing countries, far more children die from retinoblastoma, or lose their eye, than in developed countries

The causes behind this condition are unknown. It occurs equally in boys and girls. Some 60% of cases are in one eye. The cancer is either because of an arbitrary (sporadic) mutation that triggers it or is inherited. If one parent is a carrier, the individual has a 50% risk of developing the cancer. Such a cancer is called a germline cancer, and it may present as cancer in both eyes.

Retinoblastoma is not the only cancer that affects the eye; there are other kinds of intraocular tumors too, such as choroidal melanoma, choroidal hemangioma, retinal vascular tumors, and other ocular surface tumors. 

Retinoblastoma appears in very young kids. Some of the possible symptoms are:

• Leukocoria, or a white spot in the eye
• Squint or strabismus
• A swollen or painful eye

Early and timely identification of children with this condition is critical to save the affected eye and the child’s life.

The Operation Eyesight Universal Institute for Eye Cancer at LVPEI sees about 200 to 250 new retinoblastoma cases every year across our network. Treatment options include:

• Chemotherapy: Drugs that target the cancer cells are injected intravenously to shrink and ultimately destroy the cancer cells. In cases where IV chemotherapy is ineffective, our centres in Hyderabad and Vijayawada also offer intra-arterial chemotherapy (IAC). IAC a delicate and complex procedure where a concentrated dose of cancer-inhibiting medication is delivered into the patient’s ophthalmic artery, the primary source of blood to the eye. IAC is particularly effective in treating retinoblastoma, but it requires precision, skill, and the support of a multidisciplinary team. 
• Radiation therapy: LVPEI offers plaque brachytherapy, a complex procedure that places a radioactive plaque accurately on the tumor while sparing healthy tissue. After a few hours, the plaque is removed. LVPEI is one of the few institutions that can offer this therapy. The centres in Hyderabad and Vijayawada complies with Atomic Energy Regulatory Board (AERB) regulations. 
• Lasers and cryotherapies: In the early stages of the cancer, laser photocoagulation, or focused laser beams that destroys cancerous tissue may be effective. Similarly, a cryophobe is placed on the tissue using extreme cold to freeze and thaw the cells multiple times, ultimately killing them.
• Surgery: If patients present late, they may need removal of either tumor tissue, or evisceration—the eye itself to save the life of the patient. 

In some cases, trimodal therapy—all three approaches combined—may be needed to save the sight, and life, of the patient.