retinoblastoma

Retinoblastoma

Retinoblastooma is an ocular retinal cancer. Young children, usually under three years of age, become ill.

Retinoblastoon is usually suspected to be a white reflection or a less commonly known symptom of typical symptoms. Retinoblastooma is found in the eye by understudy.

The majority of patients will improve because the disease is usually not spread outside the eye at the stage of Detection. 1

Retinoblastooma is the most common eye cancer in children. Of them, even more than 95% improve their disease. [2] If the tumor occurs in only one eye, it is most often increased to a large extent before detection and a blind eye removal surgery may be necessary. If the tumour occurs in both eyes, the treatment is used, depending on the size of the tumor, e.g. laser treatment, freezing therapy, radiotherapy and chemotherapy.

Untreated retinoblastoma can cause blindness, and nowadays mainly in developing countries, death. The disease is quite often heritable, and 10% of patients have a family history of Retinoblastoomaa in the past. [

Symptoms and Signs
Illness age
Retinoblastooma, a retinal varhaissolusyöpä, is a rare intra-ocular childhood malignant tumor. There are three to five children per year in Finland, one of the eight thousand live births. In relation to fertility, the incidence of retinoblastooman throughout the world is almost as great. As its name suggests, it is an evolving retinal tumor that can sometimes exist at birth. The sufferers three-quarters are under three years old, and after the fifth age retinoblastoma not exactly meet.

Early signs
The most common first symptom of Retinoblaway is Leukobasket, which is a pale reflex reflecting in the black. It's easier to see when the black-and-dark light is large than in bright light. Today, it is often seen in a flash photograph of a child, where the black is not seen as orange but white. Leukorkori is always a subject of emergency ophthalmology examination.

The second most common symptom is a carsastus, which is caused by an accurate vision of the tumour. In Finland, the retinoblastoma hardly ever causes eye pain or redness, but in developing countries, the retinobblatootrip outside the eye is not uncommon.

Findings
Retinoblastooma is shown in the eye base study as a grey-white colony or hotplate associated with the retina. The colonies are of different sizes and often have bright calciferous. The further Ehtineelle retinopathy is characterized by the satellite of tumour cells in the vitreous humor. The retinoblastoomas under the retina can hide behind the retinal detachment. The growth method is relevant to the selection of treatment.

The Retinoblastooma is limited to quite a long period of the eye. It can spread over the optic nerve to the film-space and further into the area of the brain. Tumor cells may spread through the blood flow involved mainly in the skeletal and brain. In this case it is almost always a retinoblastooma grown in the choroidal or optic nerve. Retinoblastoomova can sometimes rarely penetrate the ventricle water involved in the venous circulation or from outside the eye to the cervical lymph nodes.

Inheritance and genetics
The Retinoblastooma is divided into clinically two main categories, a form of heritable dispersion and inherited form, which may occur by genera. In most cases, retyinoblastooma is the result of new Iturada mutation and is the first case in the family. In both cases, it is the cause of damage to the normal development of the retina, which is therefore a protective of the retinobblastoon. A possible range of disease in the inherited Retinobblastooma is considerably wider than the eye tumour itself.

Inherited retinoblastoma
A large half of the retinoblotopine is caused by two consecutive mutations occurring in an evolving retinal cell. The first inactivates the Retinoblastoomogen one copy. The remaining copy allows the retinal cells to grow and differentiate as normal. If another copy is activated before the final differentiation of the cell, the child becomes ill with the Retinoblastoon. Typically, only one cell has the undergo of both mutations. The child states one tumour colony in one eye, averaging just under 2 years of age.

As a result of mutations in the evolving retina, retinoblastoma is not inherited by succeeding generations and does not involve a specific risk to other tumours.

Hereditary retinoblastoma

Less than half of the retinoblastoomins have inherited the first mutation from their parents. All retinal cells are then susceptible to developing the retinobblastooman. Another mutation tends to occur in both eyes and at several points of the retina before the cells ripen. A hereditary retinoblastoma is typically created in both eyes, and in one eye it causes more tumors.

A mirrored retinoblastoma is diagnosed earlier than an inherited, usually under one year of age. In all cells, a hereditary retinoblastooma has one normal and one defective retinobblatoomagene. The tumor appears to be inherited as if a genetic defect would run a family history in Autosomi Dominantiti. In reality, the last copy of the normal existing gene is being inactivated.

Most of the parents of children with inherited Retinoblastoon have not been suffering from the Retinoblastoomaa, but the first mutation has occurred in a single germ cell. Their later born children do not have a particular risk of having Retinoblastoomaa. In only one tenth of the cases, the other parent is a genetic defect asymptomatic applicant, in which half of his children inherit the gene and then usually becomes ill with the Retinoblastoon.

Forms of Disease

Single Eye tumor

Individual tumour colonies are similar in appearance to Retinoblastooma and inherited form. If the disease is only in the other eye of the patient, it is heritable whenever there are at least two tumour colonies in the retina. If there is only one colony and the child is already at least a year old, the probability speaks for an inherited retinoblastoon.

Two-eye tumor

In both eyes, the retinoblastoma is always heritable. Children are diagnosed with an average of five tumour colonies and can become more after detection of the disease.

 "Three-eye " tumor

A hereditary retinoblastooma may be associated with a brain tumor resembling an eye tumour, which is usually generated by the pineal gland. Since the pineal gland in some species acts as a sensory organ, this image of the disease has been taken to be called "trilateral" or "three-eye" Retinoblastoon. Part of these intracranial retinoblastoself is located in a cuckoo, separate from the Turkish saddle. These tumors tend to spread through cerebrospinal fluid in the rest of the central nervous system area.

A vestipeed tumor

The retinoblastooma can sometimes destroy itself and leave behind only a calciferous wound. This is a rare phenomenon, and most of the retinoblatics, which are self-healing, are benign retinocytosis.

Benign Retinocytooma

Patients with hereditary retinoblastooma and their close relatives may be monitored for unchanged asymptomatic retinal tumors that resemble radiotherapy for enhanced retinoblastooma. They are considered a retinomatomogen defect as benign manifestations. The latter mutation has occurred in an almost permanently differentiated retinal cell. If the benign nature is confirmed by tissue research, the tumour is called retinytosis. Very rare, the retinoblastoomas that have been observed in adulthood are likely to have been born with the subsequent change in malignancies. Also, some children with Retinoblastooma may develop retinospytosis as an intermediate stage.

13q chromosome deficiency syndrome

Some 1% of children with inherited retinoblastoon can be observed in chromosome studies with an abnormal chromosome 13. In addition to the Retinoblastooma, there are slow mental and physical development, as well as structural abnormalities of the face and the various organs due to the damage of genes located in the vicinity of the Retinobblastoomogen.

Bone cancer and other tumors

Patients with inherited disease are at risk of developing other cancer tumours later. The most typical secondary tumour is osteosarcoma, which is most commonly observed during puberty, bone cancer, which is up to 500 times higher than normal in patients with hereditary retinoblastooma. More common than usual are also soft-eating and skin melanoma. Mixed estimates of the prevalence of these tumours have been expressed; Most of it is not.

Detection and studies

Retinoblastooma is indicated by a careful eye base examination. An echocardiogram and an MRI scan exclude growth outside the eye. They are also helpful if the tumor remains hidden behind a detached retina. The sampling is not used because of the risk of spreading it.

Premature retinal disease and congenital developmental disorders can be distinguished from the retinoblastomia with the above-mentioned studies. Coates disease where blood vessels that deviate from the veins can cause tumour-like retinal whitish fat deposits and retinal detachment, may sometimes arouse suspicion of retinoblastoon. Also, a benign retinal support for a cell tumor, astrocytooma, can sometimes resemble a retinoblotoma.

Metastases are very unusual when the tumor is established, so routine bone marrow sampling and other similar studies have been abandoned as a rule. They are appropriate if the disease is particularly suspected.

Due to the possible inheritance of the tumour, parent and sibling eye soles are always investigated.

Treatment options

The treatment of retinoblastooma in Finland is concentrated in the ophthalmology Clinic of Helsinki University Hospital.

The treatment of Retinoblastooma is an attempt to maintain a usable vision, destroy the tumor, prevent it from spreading and, if possible, avoid increasing the risk of other malignant tumors in patients with hereditary disease. Heredity counseling is also important. Genetic studies in which the inheritance of an incorrect Retinobblastoomogen can be demonstrated directly or predicted on the basis of association analyses are important in the genetic counselling.

Freezing and laser therapy

Very small tumour colonies located in the back of the eye can be destroyed by heating Infrapunalaserilla. Similarly, small tumors located in the front of the retina can be frozen through the eye sclera.

cell blockers

Chemotherapy is now commonly used to shrink the larger retinoblastine before they are permanently destroyed by laser, freezing or radiotherapy. This type of treatment is mainly used in the case of heritable form of disease. The disease in a child with an inherited retinoblaway has usually progressed so far before it is found that eye removal is often a more sensible alternative to chemotherapy that affects the whole organism. In recent years, more severe cases of outbreaks have also been used to treat local chemotherapeutic agents, such as intravitless injection.

Disc radiotherapy

If the tumour colonies are small, located close to each other and are not associated with a significant sowing of the vitreous, it is possible to treat the eye by attaching it to the outer surface of the tumor over a quantity for the radiation therapy plate. In this case, the tumour can be given a radial dose without the surrounding tissues suffering much. There are currently beta-radiation-producing ruthinium and gamma-ray-producing iodine plates that do not affect the orbital direction at all. This treatment does not increase the risk of the inherited Retinoblastoon's sufferers to obtain later radiotherapy for tumors. Disc radiation therapy is also used after chemotherapy as adjuvant therapy.

External radiotherapy

If the patient is diagnosed with tumour sowing intravitnon or tumour regeneration and other treatments have not been effective, an external radiation treatment with a traditional radiotherapy device must be used. It is an effective form of treatment, but the range of radiation from the surrounding tissues exposes other malignant tumours of the inherited disease.

Eye removal

Removal of the eye is still a sensible form of treatment in those cases where a usable vision of the eye is permanently lost, especially in the case of non-inherited disease, in which case the other eye is not at risk of contracting.

Intracranial tumour Treatment

Intracranial pineal gland or the Turkish Satulan region retinoblastoma is difficult to heal. Surgery usually does not help. Severe chemotherapy combined with bone marrow transplantation, radiotherapy or both may destroy this tumor.

Forecast

The prognosis of the currently treated retinobblastoompatients is excellent and in Finland the mortality rate in the eye tumor itself is less than 5%. One eye disease sufferers have a healthy eye and the prognosis is also excellent in terms of eyesight. The prognosis for the vision of two ocular diseases is the deterioration of subsequent new tumour colonies or the re-growth of previously treated colonies. The probability of both occurrence decreases with age.

The prognosis for hereditary retinoblastooma is worsening of tumours of the pineal, osteosarcoma and other malignant neoplasms for which they are more susceptible to genetic defects.