How can it be
detected early?
The most common time of detection of this tumor is around one year of
age, when both eyes are involved; and around two years of age when only
one eye is involved. A periodic evaluation of the child by an
ophthalmologist, especially if there is a family history of this disease
may help in picking up the tumor early. Whenever the child is suspected
to have poor vision or if there are any of the above symptoms noted, an
ophthalmologist must be consulted.
To establish the diagnosis, the doctor may need to examine the child
under general anesthesia. A few special tests like ultrasonography, CT
scan, X-rays, MRI, blood tests, spinal tap, bone marrow biopsy etc. may
be done to establish the diagnosis and to find out the extent of the
disease.
If a child has been diagnosed to have
retinoblastoma in one eye, can he also develop the same disease in the
other eye?
Yes. 25% of these tumors can affect both the eyes. The other eye must
also be evaluated thoroughly for the presence of any tumor. Also in a
few cases, the other eye may develop tumor a bit later. Therefore on the
follow up visits also, the other eye should be examined.
Is the child also at a risk for other
diseases or tumors?
Some of these children, especially those in which the tumor involves
both the eyes, are at a risk for developing other tumors like osteogenic
sarcoma (a cancerous tumor that affects the bone). The radiation therapy
for the tumor (external beam radiotherapy) increases this risk of other
tumors. Your ophthalmologist will discuss these issues with you.
My child has been diagnosed to have
retinoblastoma. Should I get my other children examined as well?
As we know, a few of these cases may be hereditary, therefore all the
siblings of the affected child must get an evaluation to rule out this
disease.
What are the chances that the next child
to be born would have the same disease?
These chances may range from as low as 1 in 15,000 to as high as 45%,
and depend upon many factors and like whether there is a family history
of such tumor, whether the tumor is involving both eyes etc. A genetic
counseling should be sought when planning for another baby. Similarly,
when a person with treated retinoblastoma plans to have a child, he or
she must also consult the doctor for genetic counseling.
Is it curable?
Yes. If detected at an early stage, the life and many times the sight
and the eye of the child may be saved. After removal of the tumor, the
chances of survival are very good in these children.
What are the treatment options?
The aim of treatment in retinoblastoma, in order of priority, is to
save the life, eye, sight and cosmesis of the child.
There are many treatment modalities for this tumor. These are:
- Enucleation: This means removal of the eye. The
eye with the tumor is removed and is sent for a pathology report.
The space is replaced by an artificial implant made of plastic,
rubber or coral. The child is fitted with prosthesis or artificial
eye, made to match the other eye. This artificial eye, of course,
does not have any vision.
- External beam radiation: as an alternative to
enucleation, this method of giving radiation treatment to the eye
may treat the tumor while preserving the eye. As the tumor is very
radiosensitive, this treatment method is very effective. Though the
radiation may cause damage to the surrounding structures of the eye,
e.g., lens (cataract), lacrimal system (dry eye), orbit (bone
maldevelopment) etc. It also increases the chance of developing
secondary tumor of the bone, especially in children with tumor
involving both eyes.
- Localized Plaque radiation therapy: This method
involves applying radioactive plaque close to the tumor on the
sclera, and thus gives the radiation therapy in a localized area. It
significantly decreases the local complications associated with
radiation therapy.
- Photocoagulation: Laser treatment for the tumor
is effective and safe for tumors that are smaller in size. In the
right cases, this treatment may help in preserving eye as well as
the sight of the child.
- Cryotherapy: this involves freezing of the
tumor by applying a special probe on the external surface of the eye
(sclera). This treatment is done under direct observation and is
effective in saving the eye and sight in patients with small tumor.
- Chemotherapy: Treatment with anticancer drugs.
There has been a recent preference towards treatment with systemic
drugs as an additional treatment. The chemotherapy may decrease the
size of the tumor and thus make it more amenable to local treatment
like laser, cryotherapy and plaque radiation.
Which treatment option is the best?
The treatment of retinoblastoma is individualized for each patient. The
treatment modality for a given child is decided based on many important
factors. These are:
- Size of the tumor
- Location of the tumor
- Whether the tumor has spread
- Status of the other eye
- Associated complications like retinal detachment, glaucoma
- Family history
- The wishes and concerns of the parents
- Availability of the facilities
As this is a difficult decision made based on many factors, it is
advised to discuss with the treating ophthalmologist about the best
treatment option for the concerned child. All the advantages and
disadvantages of each option must be weighed before taking a decision.
It is also important to discuss the rehabilitation and the cosmetic
correction after the surgery.
What is done if the child has tumor in
both eyes?
In these cases, generally the worse eye is enucleated and the other eye
is tried to salvage by using other methods of treatment. However,
unfortunately in a few cases, both eyes may have to be removed.
Why is early detection important?
As mentioned earlier, the safer and less destructive procedures may be
taken when the tumor is in its early stage. By detecting and treating
the tumor early, we may save the life, sight and eye of the child.
What are the long-term consequences of
retinoblastoma?
Even though retinoblastoma is a life threatening disease, it is rarely
a fatal one if treated appropriately. With the correct treatment in the
hands of an experienced ophthalmologist, the retinoblastoma patient has
a very good chance of living a long, full, and happy life.
It is very important to have a proper follow up with the treating
ophthalmologist for the eye tumor as well as for detection and treatment
of possible other tumors. The frequency of follow up depends upon the
type of tumor and the type of treatment given. Also in children with
only one eye left, extra care must be taken to avoid injuries to the
good eye.
