Retinoblastoma

Description:

Retinoblastoma (retina cancer) – the malignant tumor of an eye developing preferential at children's age from fabrics of an embryonal origin. The peak of a disease falls on 2 years. Almost all cases come to light to 5-year age.

Retinoblastoma symptoms:

The clinical picture of a retinoblastoma is defined by type of tumoral growth: endophytic or exophytic. With an endophytic growth the retinoblastoma develops from the cells located on the internal surface of a retina. At exophytic option - from retina periblast cells.

The majority of tumors is diagnosed when process did not go beyond an eye yet. Signs and symptoms of an intraocular tumor depend on its size and an arrangement.

One of the first symptoms of a tumor is the leykokoriya (a white pupillary reflex) or a symptom of "a cat's eye" which is quite often described by the child's parents as some unusual luminescence in one or both eyes.  This symptom appears when a tumor already rather big or in cases of tumoral stratification of a retina that leads to protrusion of tumoral weight behind a crystalline lens which are visible through a pupil. Before emergence of a leykokoriya the small tumors located close to fovea (pole) and macula (spot) lead to loss of the central sight, then and to loss of solid vision, quite often squint is a consequence of what.

Sight loss - one of early symptoms of a retinoblastoma which seldom comes to light as small children are not able to estimate its development. Squint - a symptom of development of a tumor, the second for frequency. It comes to light more often as it is noticed by people around.

Intraocular tumors are painless until secondary glaucoma or an inflammation develop. The pains caused by increase in intraocular pressure - a retinoblastoma symptom, the third on frequency.

At late diagnosis when the ekstraglazny stage of a disease already takes place, distribution of process into an orbit and signs of dissimination of process (the remote metastasises) comes to light.

Bilateral hereditary tumors in most cases have multifocal defeat with several tumoral educations in each eye whereas at sporadic cases usually there is a single tumoral node.

Current of a retinoblastoma

The intraocular stage of a retinoblastoma threatens sight. The tumor becomes a visible oftalmoskopicheska when its sizes have 0,25 mm a diameter. The tumor of this size is concluded in a retina and presented in the form of translucent hemispherical weight which darkens an oftalmoskopichesky type of the subject choroid.

The tumor grows and becomes more impenetrable, is frequent it slaborozovy color because of vessels which can be on its surface. The tumor can grow inside (endophytic type of growth) in a vitreous. Endophytic retinoblastomas usually friable, they as if metastasize in a vitreous, can extend in an anterior chamber of an eye. In the period of an intraocular phase of a disease glaucoma often develops. Glaucoma develops when the tumor reaches the big sizes, filling an eyeglobe or a tumor causes peeling of a retina. Such tumors provoke strengthening of vascularization of an iris of the eye that, it is not excluded, is result of the answer to primary ischemia. This strengthening of vascularization leads to constantly increased intraocular pressure which usually causes increase in an eyeglobe in the child. An eye becomes the blind person, the pain syndrome, vomiting, anorexia develops. Growth of a tumor can go outside for a mesh cover. Tumors with these signs of growth are called exophytic. In one eye tumors of both types of growth can be observed.

The extraocular stage of a retinoblastoma threatens not only to sight, but also the patient's life because of direct distribution on the central nervous system. Spread of a tumor on цнс usually goes on a soft meninx through lamina cribrosa which delimits an eye from a brain, and also the tumor can extend on an optic nerve, is especially frequent - by its intracranial part. Spread of a tumor on a choroid leads to hematogenous innidiation. The tumor can sprout also a sclera. In late stages of a disease regional lymph nodes are surprised and the remote metastasises appear.

Retinoblastoma reasons:

Most often the retinoblastoma is caused genetically (if the child inherits a mutant allele of a gene of Rb, then the second mutation occurring already in a retinoblast leads to formation of a tumor). Cases when at the parents who had a retinoblastoma healthy children are born, have quite small percent from total quantity of children in such families.

Treatment of the Retinoblastoma:

Now in treatment of retinoblastomas are used: operational method, beam and chemotherapy. The correct combination of the offered methods gives the chance of obtaining good results. Therefore when planning treatment the next basic moments have huge value:

    * defeat unilateral or bilateral;
    * sight is kept and there is a possibility of its preservation;
    * defeat only intraocular or process already extended to an optic nerve;
    * whether distribution of process into an orbit, on цнс, existence of the remote metastasises takes place.


Now there is a tendency to the greatest possible conservative treatment. Cryotherapy and photocoagulation - two fine methods of treatment of retinoblastomas which are allowing to keep an eye and sight and almost not giving complications. In case of a recurrence of a disease treatment repetition is possible. However conservative treatment is effective only at small tumors. Cryotherapy is shown at defeat of front department of a retina, photocoagulation - at defeat of back department of a retina.