Definition
Retinoblastoma is a malignant tumor that affects the retina. This tumor can develop at any age, but usually tends to arise during the first five years of life.
Generally, retinoblastoma affects only one eye, but the possibility that the neoplasm may develop in both eyes cannot be excluded.
Two forms of retinoblastoma can be distinguished: sporadic (60% of cases) and hereditary (40% of cases).
Causes
The triggering cause of the tumor lies in the mutation of the tumor suppressor gene RB1 located on chromosome 13. This genetic alteration causes the cells of the retina to develop rapidly and uncontrollably, thus giving rise to the neoplasm.
In some cases, the altered gene is inherited directly from the parents (hereditary form); in other cases, however, genetic alteration occurs randomly during fetal development (sporadic form).
Symptoms
The characteristic symptom of retinoblastoma is the leukocoria, that is a greyish-white reflex that is found at the pupil when the latter is illuminated by a beam of light.
Other symptoms that may occur in patients with retinoblastoma are: eye pain, redness of the eyes, eye fatigue, decreased vision, narrowed field of vision, developmental delay, glaucoma and strabismus.
Information on Retinoblastoma - Drugs for the Treatment of Retinoblastoma is not intended to replace the direct relationship between health professional and patient. Always consult your doctor and / or specialist before taking Retinoblastoma - Retinoblastoma Drugs.
drugs
Fortunately, children who are diagnosed with retinoblastoma have a good chance of recovery. In this sense, early diagnosis of the neoplasm is very important, in fact, retinoblastoma is a very aggressive tumor that can metastasize, spreading to bones, bone marrow and lymph nodes. For this reason, its timely treatment is essential, before the metastasis begins.
The therapeutic strategies undertaken against retinoblastoma, of course, aim at the eradication of the tumor, while trying - to the extent possible - to safeguard both the eye and the patient's sight.
When the tumor is in an early stage, generally, it is intervened through laser therapy, thermotherapy, or by freezing the tumor, that is through cryotherapy.
When, on the other hand, the tumor is in a more advanced stage, the doctor may decide to intervene by subjecting the patient to chemotherapy and / or radiotherapy.
Furthermore, chemotherapy and radiotherapy can also be used to treat metastatic retinoblastoma and, if necessary, to eliminate any residual tumor cells following other treatments.
Generally, chemotherapy is administered systemically, although, in some cases, it can be administered directly into the ophthalmic artery (via the arterial route), minimizing the side effects.
In the case in which the tumor is too large and other therapeutic strategies cannot be undertaken, instead, the doctor may decide to resort to surgical removal of the diseased eye, followed then by the placement of an implant in the eye socket.
Vincristine
Vincristine (Vincristina Teva ®) is an antitumor of natural origin, isolated for the first time from Madagascar periwinkle. It is an active ingredient that has therapeutic indications for the treatment of retinoblastoma.
The drug is available in pharmaceutical formulations which allow it to be administered parenterally. The vincristine dose usually used in children is 1.5-2 mg / m2 of body surface area, to be administered intravenously once a week.
However, in children whose body weight is less than 10 kg, it is generally recommended to start the vincristine-based therapy with a dosage of 0.05 mg / kg of body weight, to be administered intravenously once the week.
Cyclophosphamide
Cyclophosphamide (Endoxan Baxter ®) is an anticancer drug belonging to the class of alkylating agents, available in pharmaceutical formulations suitable for oral and parenteral administration.
When administered intravenously, the dose of cyclophosphamide usually used is 3-6 mg / kg of body weight per day.
In cases where it is necessary to establish a maintenance therapy, this is usually done by administering cyclophosphamide orally at a dose of 50-200 mg per day, to be taken with plenty of water.
However, the doctor will determine the exact amount of medicine to be taken, the frequency of administration and the duration of treatment for each patient, depending on the severity and stage of retinoblastoma and on the clinical conditions of the patient and his response to the therapy itself.
