A 4 yr old child presented with leucokoria with cataract in one eye. On fundoscopic examination he is found to have two small nodules in another eye. Which of the following is most appropriate line of management? (AIPG 2011)
|A||Wait and watch policy|
|B||Enucleation of both eyes|
|C||Enucleation of one eye (Left) and focal treatment of the nodules on another (right) eye|
(Ref. Khurana Ophthalmology 4th /280; DeVita vol1. Pg. 2056)
Bilateral retinoblastoma is currently treated with initial chemotherapy, with careful assessment of Rx response with examination under aneasthesia with each cycle………………………….Ref. DeVita, Hellman, and Rosenberg's cancer: principles & practice of cancer ...: Volume 1 - Page 2056.
1. Treatment of unilateral retinoblastoma:
a. Tumors less than four pupil diameters may be managed with radiation therapy delivered by plaques of radioactive ruthenium or iodine (brachytherapy) and cryotherapy. Larger tumors require enucleation of the eye
a. Following the diagnosis, the fellow eye should be examined with the pupil dilated every three months for five years. After that, follow-up examinations may be performed at greater intervals.
3. Clinical course and prognosis:
a. Left untreated, a retinoblastoma will eventually metastasize to the brain and cause death. Patients frequently develop a second malignant tumor such as an osteosarcoma.
b. The currently available treatment methods for retinoblastoma include intravenous chemoreduction (sometimes combined with subconjunctival chemoreduction), thermotherapy, cryotherapy, laser photocoagulation, plaque radiotherapy, external beam radiotherapy, enucleation, orbital exenteration, and systemic chemotherapy for metastatic disease.
c. Eyes with unilateral retinoblastoma are generally managed with enucleation if the eye is classified as Reese-Ellsworth group V; for those eyes in groups I to IV, chemoreduction or focal measures are used.
4. Treatment of bilateral disease:
a. For bilateral retinoblastoma, chemoreduction (carboplatin, etoposide, and vincristine - generally given for 6 cycles) is utilized in most cases unless there is extreme asymmetric involvement, with one eye having advanced disease necessitating enucleation while the other eye has minimal disease, treatable with focal methods.
b. Most children with bilateral retinoblastoma are treated with chemoreduction for at least one of their two involved eyes().
include laser photocoagulation, thermotherapy, cryotherapy, and plaque radiotherapy. Most of these therapies are employed for small tumors, especially those that have been reduced by chemoreduction. Commonly, focal therapies are applied to an eye while the child is receiving chemoreduction, and they are repeated to each tumor at each chemotherapy session. Plaque radiotherapy is generally reserved for tumors that fail other focal therapies, even those that reach a moderate size, up to 8 or 10 mm in thickness. The remainder of the focal therapies are reserved for small tumors, generally those under 3 mm in greatest dimension. Laser photocoagulation is usually employed for small retinoblastomas posterior to the equator of the eye.
External Beam Radiotherapy
Retinoblastoma is generally a radiosensitive tumor. External beam radiotherapy is a method of delivering whole eye irradiation to treat advanced retinoblastoma, particularly when there is diffuse vitreous seeding.
Enucleation is a frequently used and important method for managing retinoblastoma. If there is advanced disease with no hope for useful vision in the affected eye or if there is a concern of invasion of the tumor into the optic nerve, choroid, or orbit, then enucleation is appropriate. Those eyes with secondary glaucoma, pars plana seeding, or anterior chamber invasion are also generally best managed with enucleation.