Retinoblastoma - a malignant tumor of the eye, which develops mostly in childhood from the tissues of embryonic origin. The peak of the disease accounts for 2 years. Almost all cases are detected before the age of 5. The prevalence of retinoblastoma small - about 1 in 20,000 newborns. In Europe the disease occurs almost 4 times less than in Asia.
Retinoblastoma can be: one-sided two-sided.
Bilateral form of the disease occurs in about one in four patients and in most cases is hereditary.
Unilateral tumor is usually non-hereditary, but there is a clear link diseases with chromosomal aberrations.
As a rule, it is a deletion of part of the long arm of one chromosome 13 gene pairs with damage to RB1. This corruption is typical for retinoblastoma, as well as for osteosarcoma. It is believed that retinoblastoma develops from the neuroectoderm of the retina. At the same time tumor cells have different degrees of differentiation. For larger tumors characterized by foci of necrosis and calcifications. Retinoblastoma is represented by small undifferentiated cells with large nucleus. Typical of multiple foci of tumor on the retina. Most patients die of metastases in the CNS of the optic nerve, and bone metastases and bone marrow to the blood.
Clinic of retinoblastoma.
One of the first symptoms of retinoblastoma is a "cat's eye" or leykokoriya. Unfortunately, this symptom occurs when a sufficiently large size of the tumor. Small tumors of the central location can until leykokorii lead to loss of vision in the central part, the loss of binocular (by volume) of view, the development of strabismus. For a long time, the tumor can be "silent", but with the development of secondary glaucoma and inflammation there is pain.
So, for the first piznakam disease include: Loss of vision. One of the earliest, but poorly detectable symptoms in young children Strabismus. Pain. It occurs due to increase in intraocular pressure during the growth of the tumor.
Other symptoms commonly associated with tumor metastasis. Hereditary bilateral eye lesions are most dangerous, because tumor at the same time, usually multiple. With the growth of endophytic (inside the eye) often develop glaucoma and pain. In the future, joins blindness. For exophytic growth (outside the eye) the danger is quite near metastasis to the central nervous system and the formation of distant metastases.
Diagnosis of retinoblastoma.
Usually held bilateral eye examination under general anesthesia (under anesthesia). Widely used methods of medical imaging:
X-ray computed tomography (CT, CT)
magnetic resonance imaging (MRI).
All three methods are used quite extensively and mutually reinforcing. They allow a detailed characterization of the tumor in the eye, and evaluate the extent of the process. A biopsy (puncture) of the eye tissue is not carried out because of the high risk of artificial metastasis of tumor cells in this procedure, but the red bone marrow biopsy and lumbar puncture performed often enough. In all patients examined cerebrospinal fluid and bone marrow.
Treatment of retinoblastoma
In most cases, the radical, ie removal of the eye is carried out. However, in recent years, there chemoradiotherapy methods that often carry Organ treatment. This avoids the mutilation, such as enucleation (eye removal), and even more radical orbital exenteration (removal of the entire contents of the orbit). Also used radiokobaltovye applications, photocoagulation, cryosurgery and other methods.