Retinoblastoma : Presentation, Diagnosis, Treatment, Complications
The treatment options of retinoblastoma requires a great deal of expertise of the response of the disease at its various stages, and the potentials of each treatment option. Treatment has undergone paradigm of change along the past 50 years. From a near fatal disease, it now has a 95% survival. The current management options target organ salvage and vision salvage. The current treatment guidelines are set as per the International Retinoblastoma Classification and TNM classification of the tumor and are followed uniformly across the globe.

1.Chemotherapy: Systemic chemotherapy is now the mainstay and generally the 1st line of management. It aims at reducing the size of the tumours (chemoreduction) to allow for consolidation treatment with other agents to eradicate the tumours. Depending on the response to chemotherapy, other modalities are used in conjunction. Drugs used are Vincristine, Etoposide, carboplatin, cyclosporine .

2. Sequentially aggressive local treatment (SALT) :
This includes trans pupillary thermotherapy( heating the Tumor) , retinal lasers , cryotherapy (freezing the Tumor) and may be used alone in very small tumors or in conjunction with chemotherapy for larger ones. These technique targets organ as well as vision preservation.

Regional chemotherapy: Intravitreal chemotherapy (melphalan) may be given in selective cases . Periocular topotecan is another modality used. Recently, supra selective chemotherapy administered via the ophthalmic artery is being increasingly used across the centres . This technique avoids systemic side effects of chemotherapy and avoids removal of the eye as well. This technique has been aptly christened "chemo surgery”. Melphalan, carboplatin and topotecan have been used for this technique

3. Enucleation: Upto 25% -50% of the eyes may need eye removal or enucleation depending on the severity of the disease. Getting a long optic nerve stump (atleast 15 mm) is very important, so is proper histopathology to confirm the tumor extent.A ball like orbital implant is placed in the socket during primary surgery. A prosthetic eye can be fitted as early as 6 weeks post operative with excellent outcomes.(FIGURE 3: Post enucleation prosthesis) Generally, these procedures don't interfere with any subsequent Tumor treatment modalities.

4: Radiotherapy for retinoblastoma has a limited role in the current day scenario. It is generally resorted to in chemo resistant cases. It can be in the form of plaque brachytherapy for unifocal medium sized lesion or external beam radiation for multifocal disease with vitreous seeds.

Figure 3: Right eye ocular prosthesis after enucleation surgery and orbital implant




Contributor Information and Disclosures

Himika Gupta
M.S., DNB.


First Created : 5/7/2015

References

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