Retinoblastoma - a intraocular malignancy that develops from neuroectodermal retinal tissue and occurs mainly in young children.When retinoblastoma patients lose central and binocular vision, they develop leykokoriya, strabismus, proptosis, secondary inflammation and glaucoma.
a rigorous arrangement of intraocular tumor patients recover 90%.But late diagnosis of retinoblastoma starts to progress and as a result there are difficulties in treating these patients.
main peak of retinoblastoma accounts for two-year age.And already to five years revealed all kinds of diseases.
tumor diseases in the form of retinoblastoma in 10% of children associated with pathological abnormalities in chromosomes, while other patients are characterized by functional and structural changes in the RB1-gene, which is typical for an autosomal dominant mode of inheritance.When retinoblastoma family forms the gene is damaged in all somatic cells, organs, so there is a certain
Retinoblastoma 60-70% can occur sporadically in some gene mutations RBI, t. E. De novo, which is in the cells of the retina.For the initial changes characteristic deformation is occurring only in one of the cells of the retina.In the history of the patient's family are not marked tumor, so it is considered to be one-sided and a single center.Thus, these parents there is a small risk in the birth of the next child with retinoblastoma.Today, scientists geneticists suggest that the formation of tumors can occur two mutations.A characteristic pathology of sporadic initial change in every cell of the body.To re-localization of tumors characterized by mutations in the retina of the eye.
On average, 30% of patients with retinoblastoma will inherit the disease.Then, this pathology is bilateral in nature, and it can occur in any family member.In a healthy human gene that protects the eyes from the formation of retinoblastoma it is in the thirteenth and fourteenth pairs of chromosomes.As a result of the partial restructuring of the loss or alleles in identical chromosomes is the development of retinoblastoma.
Patients who inherit the disease, have all the prerequisites for the appearance of the malignant disease in the body.In conducting research on these mutations, chromosomal analysis using at the moment, you can either exclude or confirm the emergence of a hereditary factor retinoblastoma.Thus, the exact causes of the development of this pathology of the disease is not found.Presumably they can be also attributed some risk factors, among which are the food with GMOs and harmful ecological state of the natural environment.
Clinical symptoms of retinoblastoma develops the type of abnormal growth, among which are two types: endophytic and exophytic.
endophytic growth for retinoblastoma disease characterized by the development of the cells, which are located on the inside of the retina.For exophytic embodiment - on the outer surface.
basically the vast majority of tumors are detected before the disease process outside of the eyeball.All the symptoms and signs of intraocular retinoblastoma depend on the location and its size.
first clinical manifestation of retinoblastoma is the appearance of white pupillary reflex or leykokorii (cat's eye), having at the same time a kind of glow in the eyes of patients.For this symptom is characterized by large size of the tumor or in the tumor has a bundle of the retina that causes protrusions malignant mass that is localized for the lens and is visible through the pupil.Slight swelling, before the development of leykokorii, which are located in the fovea (fovea) and spot (macula), can cause loss of central vision in the beginning, then the binocular, and eventually lead to strabismus.
The earliest clinical sign of retinoblastoma is a loss of vision.However, he revealed quite rare, because of the impossibility of small children appreciate this growing defect.
More often possible to determine such a strong symptom of diseases such as strabismus, as it is clearly noticeable to others.Before the advent of increased intraocular pressure, the patient does not experience pain from the tumors in the eye.However, after the development of secondary glaucoma or inflammation, pain symptom is ranked third in the clinical picture of retinoblastoma.
When diagnosing the disease in the later stages when developing ekstraglaznaya pathology, neoplastic process extends to the orbit and metastases penetrate into remote places on the primary site.
for bilateral hereditary tumors is mainly characterized by multifocal lesions of each eye, and for sporadic inheritance - a single defeat in a tumor site.
Intraocular retinoblastoma flow is a serious threat to vision.In ophthalmoscopic examination tumor is visible only when the diameter reaches a size of 0.25 mm.As such, it is within the retina of the eye and has a transparent hemispherical mass obscuring the choroid.
With the progression of retinoblastoma tumor continues to grow and becomes impenetrable, while having a slightly pink color of the vessels located on its surface.Upon germination of the tumor into the vitreous body, it is characterized by endophytic retinoblastoma type growth.Such malignancies are kind of loose and spread inside the body and then move to the front of the eye.
glaucoma is often formed during intraocular retinoblastoma phase.This occurs when the tumor size increases to significant and completely covers an eyeball, and if retinal detachment occurs.
type endophyte retinoblastoma provoke an increase in the formation of new blood vessels in the iris, which results in primary ischemia.This increased vascularization is a consequence of constantly elevated intraocular pressure, which leads to an increase of the eyeball in the child.To further develop blindness, pain, vomiting, and there is, in the future, anorexia.
With the growth of retinoblastoma over the outer shell of the retina revealed all the signs of exophytic type of disease.For one eye may develop both types of malignant tumors.
with extraocular stage Retinoblastoma can affect not only your eyes, but also spread to the central nervous system, threatening the patient's life.In this case, the tumor penetrates the pia mater and extends to the optic nerve by intracranial portion.The progression of the malignant process of the choroid is the cause of hematogenous metastasis and its germination in the sclera.In the later stages of retinoblastoma occurs defeat the regional lymph nodes to distant metastases.
This disease is presently exists a certain classification, but, nevertheless, retinoblastoma has four stages proposed by US experts.
At the first stage of the tumor only affects the retina;while the second - is limited to the defeat of the eyeball;in the third - extraocular retinoblastoma has spread to local or regional lesions;while the fourth - a tumor metastasizes to distant places.
But since such a classification does not show all the important aspect of the tumor process, there are important prognostic options lesions retinoblastoma.Among them are the tumor within the eye, optic nerve injury and orbital tissues and also metastasize to other somatic organs.
to modern methods of treatment of retinoblastoma include: chemotherapy, radiation exposure and surgery.With the right combination of these methods it is possible to obtain positive results.Thus, to select a particular type of treatment allow for many points in the disease.As a rule, pay attention to the type of tumor lesions (unilateral or bilateral), whether to save the eyesight or not, there are only intraocular lesions or disease process affected the optic nerve.And whether or not spread to the central nervous system in orbit or have distant metastases.
At the moment, trying to make the most conservative method of treatment.For the treatment of retinoblastoma remarkable techniques used - it photocoagulation and cryotherapy, which allow you to save the sight and the eyes themselves with virtually no complications.Also, if after the course of relapse, it is possible to re-treatment.However, such conservative treatment methods are effective in small malignant tumors.Cryotherapy is prescribed to patients with lesions of the anterior retinal photocoagulation and - rear division.
One of the most frequent operative treatment of retinoblastoma is enucleation.It is performed with massive intraocular lesions;glaucoma, which was formed as a result of the proliferation of vessels retinoblastoma;while blindness and the inability to restore the lack of vision.
enucleation is the best treatment for retinoblastoma, a survival rate of positive results.The basis of this surgery include issechenie optic nerve as far as possible.And after six weeks prescribed prosthetics.Following the enucleation, mortality is minimized, and the subsequent regular inspections are carried out without the use of anesthesia.But since such operations do before the age of three, they entail a range of cosmetic defects in the absence of the eye and the strong growth of the bones of the orbit.
After removing the eyes conduct a thorough histological examination.The main determinants of poor prognosis are considered invasive extrascleral, optic nerve and choroid.In the propagation of the tumor beyond the prescribed eye orbit exenteration.To save
view retinoblastoma patients, radiotherapy method is selected.Basically, in this disease the method of external irradiation with two lateral fields.When multiple lesions in one eye (or two) when the tumors are multifocal, to irradiate the entire retina.In such radiotherapy in the irradiated field include vitreous and optic nerve.Beam radiation consists of higher doses of 35 Gy to the first and second groups 45 Gy - the third and fourth.In order to eliminate the development of cataracts after exposure, it is recommended to use special units for the lens.
This tumor as retinoblastoma, sensitive to radiation exposure and 75% of patients receiving it, can be cured.And in combination with cryotherapy this may increase the percentage of patients.After the application of surgical treatment and radiotherapy cure occurs in 85%.
chemotherapeutic treatment for retinoblastoma includes several cytotoxic drugs, among which the carboplatin, vincristine and Vepeside - is one of the most effective combinations at this time.The second group consists of the drugs cyclophosphamide, doxorubicin and vincristine.Recently, however, preference is given to the first scheme.This is because Cyclophosphamide promotes development of secondary tumors or causes infertility patients.
Over the past decade in many countries in the timely and correct diagnosis, retinoblastoma can not identify at an earlier stage, which allows the use in the treatment of only cryotherapy, photocoagulation and radiation exposure.