Rare Cancers In Childhood - Retinoblastoma

Retinoblastoma is an uncommonly rapid spreading cancer arises from cells of eye of retina, the light-detecting tissue of the eye. It is rare caver in childhood, in US approximately 300 children are diagnosed with retinoblastoma each year. About 3 out of 4 children with retinoblastoma have a tumor in only one eye and 1 in 4, both eyes are affected. Although the cancer can develop quickly, can be cured in most cases. 90% of Retinoblastoma diagnosed within the first three years of the child's life.

Types of retinoblastoma
1. Hereditary RetinoblastomaIs caused by the mutation of gene Rd at birth. it can affect not only the cells of the eye, but in all of the body's cells- If multiple tumors are present in both eyes, the term assigned is multi-focal retinoblastoma. Retinoblastoma effects approximately 70% of patient in all cases.
2. Sporadic Retinoblastoma
Retinoblastoma is caused abnormal cells growth in the retina other than hereditary .

Causes and risk factors
1. Gene
Retinoblastoma is caused by changes in or absence of a gene called RB1 located on chromosome 13q14 resulting in the inability of RB1 to produce a tumor suppressor protein that normally helps to regulate the cell cycle of cells of the retina, causing retinoblastoma. Chikdren with inherited retinoblastomas are more likely to be bilateral.
2. Radiation therapy
Children who had radiation to treat other cancer are at increased risk of retinoblastoma.
3. Family history
Increased risk of retinoblastoma if you have a family history of the disease.
4. Age
The cancer tends to develop in children before age of 3.
5. Etc.

Symptoms

1. Abnormal appearance of the pupil
The pupil located in the center of the iris of the eye that allows light to enter the retina. In retinoblastoma, the cells of the retina have become cancerous, causing abnormal appearance of the pupil.
2. Leukocoria
Leukocoria is an abnormal white reflection from the retina of the eye caused by abnomal cells growth in the retina.
3. Deterioration of vision
4. Faltering growth or delayed development.
5. A red and irritated eye
6. Cross-eyed
7. Eye enlargement
8. Etc.

Stages
Stages of Retinoblastoma depends to the size and location of the tumor.
1. Intraocular retinoblastoma
Cancer is inside one or both eyes and has not spreading to the surrounding tissue and nearby lymph node.

2. Extraocular retinoblastoma
Cancer has spread to the surrounding tissue, lymph node and distant parts of the body

Diagnosis and tests
After recording the family history and complete physical exams including eye exam with with dilated pupils to allow the doctor to look through the lens and pupil to the retina.
1. Eye exams
If the child has some of the above symptoms, the first test which you doctor order is the eye exam with a ophthalmologist including including the retina and the optic nerve with the use of general anesthesia.
1. Blood test
Blood test to check for the nonavailability of the RB1 gene.

2. Ultrasound
Ultrasound allows your doctor to visualize and assess the size and to check for any abnormality and surrounding area with image taken from the test.

3. CT Scan (computerized tomography)
A CT scan generates a large series of two-dimensional X-ray images taken around a single axis of rotation, to create a three-dimensional picture of the inside of the body in details.The pictures are viewed by your doctor to see the extent of the tumors abnormalities, such as spreading of cancer to the nearby structure and lymph nodes.

4. MRI scan (magnetic resonance imaging)
MRI (magnetic resonance imaging) is one of many advanced technology used to visualize internal structures cross sectional imaging of your body used effectively in providing the better details of the metastasis of cancer in the lymph nodes and surrounding areas.

5. Etc.

Preventions
If your family carry a genetic passing through of mutation of gene RB1, it is for the best interest of the child to have a genetic testing to assess the risk of retinoblastoma, so it can be diagnosed early for cure and with preservation of the child vision.

Treatments
1. Surgery
a. The aims of surgery is to cure and to preserve vision, if the tumor small. Otherwise, surgery is to remove as much of cancer as possible. Sometimes the tumor is very large and the vision in the eye is lost, enucleation may be necessary.
b. Risks and side effects
Surgical and anesthesia risks

2. Radiotherapy
a. Sometime doctors give radiation for retinoblastoma after surgery and before chemotherapy. In retinoblastoma Radiation may be used as treatment of retina with photocagulation with the use of high-energy x-rays or other types of radiation, radiation therapy kills the cancer cells and keep them from growing or regrowing.
b. Side effects
b.1. Fatigue
b.2. Chest pain
b.3. Heart problem
b.4. Short of breath
b.5. Skin discoloration or pinkness, irritation.
b.6. Etc.although there will be some effect on the surrounding tissue. Radiotherapy for retinoblastoma is usually used in situations when other treatments have not been successful.

3. Chemotherapy
a. If the cancer has spread, chemotherapy with use of drugs to cure retinoblastoma even the cancer has spread to distant part of the body.
Chemotherapy is most use to treat with advance stage of cancer combined with radio therapy. Chemo is recommended to shrink the tumor if surgery is not possible.
b. Side effects
b.1. Nausea
b.2. Vomiting
b.3. Hair loss
b.4. Fatigue
b.5. Anemia
b.6. Mouth sores taste and smell changes
b.7. Infection
b.8. Etc.

4. Photocoagulation
a. In retinoblastoma, photocoagulation is used to destroy abnormal blood vessels with aim to treat tumors in the eye by using a laser which produces light in the visible green wavelength that can be absorbed by hemoglobin in red blood cells.
b. Risks and side effects
b.1. Loss of vision, worsening visual acuity,
b. 2. Reduced night vision, and
b.3. Hemorrhaging in the eye
b.4. Etc.

5. Etc.
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