While its mortality rate was 100% in the early 19th century, it has now become a treatable disease with the development of early diagnosis and treatment methods. The presence of the tumor in one or both eyes, its location, size, degree of differentiation, tumor spread to the optic nerve, choroid, orbit, and the presence of regional or distant metastases are factors that affect vision and survival prognosis.
Retinoblastoma occurs approximately once in 20 thousand live births. It is a tumor of early childhood. It is most common between the ages of 1-3. 80% of children are under 3 years old and 95% are under 5 years old. It is rare to be seen after the age of six.
The two most common findings in retinoblastoma are leukocoria (white pupil) and strabismus. Leukocoria is when light is sent from the pupil to the eye, the reflection of the pupil, which should normally be red, shines white.
The red pupillary test, which should be performed by pediatricians and ophthalmologists in babies, should be performed through a dilated pupil and scanning all viewing directions, and should be performed especially in children under 6 months of age. The fact that the red reflection seen in the pupil in photographs taken with flash is not seen in the tumor-bearing eyes of these children should be an important warning for families.
Systemic Examination:
It should be done by a pediatrician and a pediatric oncologist. Systemic examination is important in children where it is difficult to diagnose retinoblastoma, in terms of tuberculosis, rubella syndrome, other systemic and metastatic diseases, and 13q deletion syndrome, which is frequently seen with retinoblastoma.
Diagnostic Tools:
In the systemic evaluation, in addition to the physical examination of the child, it may be necessary to perform some or all of the examinations such as blood count, blood biochemistry, bone marrow, Cerebrospinal Fluid, bone scintigraphy, hearing tests and kidney functions, orbital Ultrasonography (USG), brain and orbital CT or MRI.
Clinical process:
Retinoblastoma can fill the inside of the eyeball in approximately 6-8 months. In retinoblastomas that are diagnosed late or whose treatment is delayed, the tumor is found to have spread to surrounding tissues and even distant metastases. The main ways the tumor exits the eyeball are the optic nerve, choroid-sclera and limbus. The most common route of spread is the optic nerve. It can spread to the brain along the optic nerve.
Computed tomography (CT) and High-resolution CT are the most important tools used in diagnosis. Magnetic Resonance Imaging (MRI) shows soft tissue better than CT.
Differential diagnosis:
Calcification, which is rarely seen in diseases such as Coats disease, astrocytic hamartoma, and optic nerve drusen in children under 5 years of age, may lead to an error in diagnosis. Because many benign lesions resemble retinoblastoma, they are called pseudoretinoblastoma.
Treatment:
Retinoblastoma should be treated and followed up with a multidisciplinary treatment approach, aiming to preserve visual acuity and the eyeball under all possible conditions. Treatment methods: chemotherapy / chemoreduction, focal treatments (cryotherapy, thermotherapy, radioactive plaque), external beam radiotherapy, when these are not sufficient or in case of extensive or life-threatening conditions, enucleation (removal of the eye) and exenteration (removal of the entire eye organ and orbital structures, including the eyelids). removal) stop.