Retinoblastoma medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2] Simrat Sarai, M.D. [3]
Overview
The optimal therapy for retinoblastoma depends on the stage at diagnosis. Systemic chemotherapy via carboplatin, etoposide, and vincristine (CEV) is the most common regimen used to treat retinoblastoma.
Medical Therapy
- The priority of retinoblastoma treatment is:[1]
- Save the life of the child
- Preserve vision
- Minimize complications or side effects of treatment.
- The exact course of treatment will depend on the individual case and will be decided by the ophthalmologist in discussion with the pediatric oncologist.
- Children with the involvement of both eyes at diagnosis usually require multimodality therapy (chemotherapy and local therapies).
- The choice of therapy depends upon several factors such as:[2]
Systemic Chemotherapy
- Systemic chemotherapy has become the forefront of treatment in the past decade, in search of globe preserving measures and to avoid the adverse effects of radiation therapy.[3]
- The common indications for systemic chemotherapy include:
- Unilateral intraocular retinoblastoma with high risk feature
- Bilateral intraocular retinoblastoma
- Prophylaxis against metastasis following enucleation in the presence of histopathologic high-risk features
- Extraocular retinoblastoma with local and/or regional spread
- Metastatic retinoblastoma with or without CNS involvement
- Trilateral retinoblastoma
Chemotherapy Regimen
- The most common chemotherapy regimen for retinoblastoma is the combination of the followings:[4][5][6]
- This combination may be used with different dose of medication and frequency depending on the stage of the tumor.
- Usually, treatment regimen comprised of either three sessions of high dose regimen or six sessions of low dose regimen.
- Low dose regimen:
- Carboplatin 18.6 mg/kg IV q24h for one day and be repeated every (21-28) days
- Etoposide 5 mg/kg IV q24h for two days be repeated (21-28) days
- Vincristine 0.05 mg/kg IV q24h for one day and be repeated every (21-28) days
- High dose regimen:
- Carboplatin 28 mg/kg IV q24h for one day and be repeated every (21-28) days
- Etoposide 12 mg/kg IV q24h for two days and be repeated every (21-28) days
- Vincristine 0.05 mg/kg IV q24h for one day and be repeated every (21-28) days
- The above afromentioned dosage is recommended for orbital retinoblastoma and is subject to change for other type of tumor spread.
Intra-arterial Chemotherapy
- Chemotherapeutic drugs are administered locally via a thin catheter threaded through the groin, aorta, and the neck directly into the optic vessels.
Other interventions
- Other treatment modalities which are currently available for retinoblastoma include:[7]
External beam radiotherapy
- The feasibility of external beam radiotherapy depends on the stage of retinoblastoma at the time of diagnosis.
- The globe preservation rate with this method varies according to the stage of the tumor at the time of diagnosis:
- Groups I–II eyes:95%
- Groups IV and V eyes:50%
- This method is indicated as salvage therapy for seeding following unsuccessful chemotherapy, however, the decision to use this method of treatment must be made on a case by case basis.
- The dose to target tumor cells of retina ranges from 3,600 to 4,500 cGy administered in 180–200 cGy daily fractions 5 times/week.
- Contraindications of EBRT:
- Children less than 1 year of age due to the risk secondary malignancies
- For eyes with dismal visual potential
- In primary therapy of retinoblastoma
Brachytherapy
- Indications:
- Primary treatment
- Residual tumor following photocoagulation/thermotherapy/thermo-chemotherapy/cryotherapy
- Tumor recurrence
- Tumor less than 15 mm in diameter
- Contraindication:
- Presence of diffuse vitreous seeding
Thermotherapy
- Indications:
- Tumors not more than 3 mm in diameter, with no and traction, retinal vascular occlusion evidence of seeding, and located posterior to the equator
- Primary treatment
- Consolidation treatment
- Tumor recurrence
Thermochemotherapy
- Indications:
- Tumors not more than 12 mm in diameter with no evidence of seeding, and located posterior to the equator
- Consolidation treatment
Laser photocoagulation
- This method is indicated for intraocular retinoblastoma and one of the following factors:
- Consolidation of large tumors after systemic chemotherapy
- For the treatment of small peripheral or posterior tumors as the sole modality
- For the eradication of small tumor recurrences following chemotherapy and/or radiotherapy
- This method is suitable for treating tumors with the following characteristics:
- 3.0 mm or less in diameter
- 2.0 mm or less in thickness
- Without seeding into the adjacent vitreous
Cryotherapy
- Indications:
- Tumors not more than 3 mm in diameter with no evidence of seeding, and located anterior to the equator
- Primary treatment
- Consolidation treatment
- Tumor recurrence
Management Options of Retinoblastoma
Treatment options for Intraocular tumor[8] | |
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Unilateral retinoblastoma |
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Bilateral retinoblastoma |
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Cavitary retinoblastoma |
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Progressive or recurrent intraocular retinoblastoma |
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Treatment options for Extraocular tumor[8] | |
Orbital and locoregional retinoblastoma | |
CNS disease |
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Trilateral retinoblastoma |
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Extracranial metastatic retinoblastoma |
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Progressive or recurrent extraocular retinoblastoma |
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References
- ↑ Retinoblastoma Treatment. National Cancer Institute(2015) http://www.cancer.gov/types/retinoblastoma/hp/retinoblastoma-treatment-pdq#section/_53 Accessed on October 13, 2015
- ↑ Shields CL, Shields JA (1999). "Recent developments in the management of retinoblastoma". J Pediatr Ophthalmol Strabismus. 36 (1): 8–18, quiz 35-6. PMID 9972509.
- ↑ Venkatramani, Rajkumar; Jubran, Rima Fuad (2015). "Retinoblastoma: Intravenous Chemotherapy": 119–127. doi:10.1007/978-3-662-43451-2_11.
- ↑ Rodriguez-Galindo, Carlos; Wilson, Matthew W.; Haik, Barrett G.; Merchant, Thomas E.; Billups, Catherine A.; Shah, Nirali; Cain, Alvida; Langston, James; Lipson, Mindy; Kun, Larry E.; Pratt, Charles B. (2003). "Treatment of Intraocular Retinoblastoma With Vincristine and Carboplatin". Journal of Clinical Oncology. 21 (10): 2019–2025. doi:10.1200/JCO.2003.09.103. ISSN 0732-183X.
- ↑ Gallie BL, Budning A, DeBoer G, Thiessen JJ, Koren G, Verjee Z, Ling V, Chan HS (November 1996). "Chemotherapy with focal therapy can cure intraocular retinoblastoma without radiotherapy". Arch. Ophthalmol. 114 (11): 1321–8. PMID 8906022.
- ↑ Dunkel IJ, Lee TC, Shi W, Beaverson KL, Novetsky D, Lyden D, Finlay JL, McCormick B, Abramson DH (October 2007). "A phase II trial of carboplatin for intraocular retinoblastoma". Pediatr Blood Cancer. 49 (5): 643–8. doi:10.1002/pbc.21163. PMID 17301956.
- ↑ Kim, Jonathan W.; Murphree, A. Linn; Singh, Arun D. (2015). "Retinoblastoma: Treatment Options": 89–107. doi:10.1007/978-3-662-43451-2_9.
- ↑ 8.0 8.1 "Retinoblastoma Treatment (PDQ®)—Health Professional Version - National Cancer Institute".