Retinoblastoma is a rare type of eye cancer that usually develops in early childhood, typically before the age of 6. This form of cancer develops in the retina, which is the specialized light-sensitive tissue at the back of the eye that detects light and color. Retinoblastoma is diagnosed in about 250-300 children per year in the United States. It accounts for less than 4 percent of all cancers in children younger than 15 years.
In most children with retinoblastoma, the disease affects only one eye (unilateral). However, one out of three children with retinoblastoma develops cancer in both eyes (bilateral). The most common first sign of retinoblastoma is a visible whiteness in the pupil called "cat's eye reflex" or leukocoria. This unusual whiteness is sometimes noticeable in photographs taken with a flash. Other signs and symptoms of retinoblastoma can include crossed eyes or eyes that do not point in the same direction (strabismus); persistent eye pain, redness, or irritation; and blindness or poor vision in the affected eye(s).If retinoblastoma spreads, it can spread to the lymph nodes, bones or the bone marrow. Rarely, it can involve the central nervous system (CNS).
It is not exactly understood why retinoblastoma occurs. In some of the cases this cancer is caused by an RB1 gene mutation or an abnormality in chromosome 13, in which a piece of the chromosome is nonfunctional or missing. In 40% of the cases, the abnormality is present in every cell of the body including the eye and in 60% of cases, the abnormality is only found in the eye. Chromosome 13 is responsible for controlling retinal cell division. In children with retinoblastoma, retinal cell division continues unchecked, causing the retinal tumor(s).
*Retinoblastoma accounts for less than 4% of all cancers in children younger than 15
*250-300 children are diagnosed with Retinoblastoma each year, nationwide
*40% of children with Retinoblastoma will have tumors occur in both eyes (Bi-Lateral)
*30% will have the gene mutation
*5% of Bi-Lateral cases will have a visible chromosome 13 deletion
In most children with retinoblastoma, the disease affects only one eye (unilateral). However, one out of three children with retinoblastoma develops cancer in both eyes (bilateral). The most common first sign of retinoblastoma is a visible whiteness in the pupil called "cat's eye reflex" or leukocoria. This unusual whiteness is sometimes noticeable in photographs taken with a flash. Other signs and symptoms of retinoblastoma can include crossed eyes or eyes that do not point in the same direction (strabismus); persistent eye pain, redness, or irritation; and blindness or poor vision in the affected eye(s).If retinoblastoma spreads, it can spread to the lymph nodes, bones or the bone marrow. Rarely, it can involve the central nervous system (CNS).
It is not exactly understood why retinoblastoma occurs. In some of the cases this cancer is caused by an RB1 gene mutation or an abnormality in chromosome 13, in which a piece of the chromosome is nonfunctional or missing. In 40% of the cases, the abnormality is present in every cell of the body including the eye and in 60% of cases, the abnormality is only found in the eye. Chromosome 13 is responsible for controlling retinal cell division. In children with retinoblastoma, retinal cell division continues unchecked, causing the retinal tumor(s).
*Retinoblastoma accounts for less than 4% of all cancers in children younger than 15
*250-300 children are diagnosed with Retinoblastoma each year, nationwide
*40% of children with Retinoblastoma will have tumors occur in both eyes (Bi-Lateral)
*30% will have the gene mutation
*5% of Bi-Lateral cases will have a visible chromosome 13 deletion