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Retinoblastoma Treatment (PDQ®)

Health Professional Version
Last Modified: 12/06/2013

Treatment Option Overview

Treatment planning by a multidisciplinary team of cancer specialists, including a pediatric oncologist, ophthalmologist, and radiation oncologist, who have experience treating ocular tumors of childhood is required to optimize treatment outcomes.[1] Evaluation at specialized treatment centers is highly suggested before the initiation of treatment in order to improve the likelihood of ocular salvage.

The goals of therapy are threefold:

  1. Eradicate the disease to save the patient's life.
  2. Preserve as much vision as possible.
  3. Decrease risk of late sequelae from treatment, particularly subsequent neoplasms.

The type of treatment required depends on both the extent of the disease within the eye and whether the disease has spread beyond the eye, either to the brain or to the rest of the body.[2] Eyes with glaucoma and those in which glaucoma resulted in buphthalmia are significantly associated with high-risk pathology and the occurrence of microscopically residual tumor.[3] Enucleation is reserved for patients with advanced unilateral intraocular disease with no hope for useful vision in the affected eye. Subsequent risk of extraocular recurrence may be increased in the presence of high-risk histopathologic features such as massive choroid invasion, scleral invasion, and optic nerve invasion.[4-6]; [7][Level of evidence: 3iiDi]

Routine bone marrow biopsy and lumbar puncture are not indicated, except when there is a high level of suspicion that the tumor has spread beyond the globe.[8-10]

It is not uncommon for patients with retinoblastoma to have extensive disease within one eye at diagnosis, with either massive tumors involving more than one-half of the retina, multiple tumors diffusely involving the retina, or obvious seeding of the vitreous. For those with bilateral disease, systemic therapy may be used to treat the more severe eye.[11,12]

Metastasis from retinoblastoma generally develops within 1 year of diagnosis. If there is no metastatic disease by 5 years after treatment, the patient is generally considered cured.[13]

References
  1. Chintagumpala M, Chevez-Barrios P, Paysse EA, et al.: Retinoblastoma: review of current management. Oncologist 12 (10): 1237-46, 2007.  [PUBMED Abstract]

  2. Kopelman JE, McLean IW, Rosenberg SH: Multivariate analysis of risk factors for metastasis in retinoblastoma treated by enucleation. Ophthalmology 94 (4): 371-7, 1987.  [PUBMED Abstract]

  3. Chantada GL, Gonzalez A, Fandino A, et al.: Some clinical findings at presentation can predict high-risk pathology features in unilateral retinoblastoma. J Pediatr Hematol Oncol 31 (5): 325-9, 2009.  [PUBMED Abstract]

  4. Cuenca A, Giron F, Castro D, et al.: Microscopic scleral invasion in retinoblastoma: clinicopathological features and outcome. Arch Ophthalmol 127 (8): 1006-10, 2009.  [PUBMED Abstract]

  5. Gupta R, Vemuganti GK, Reddy VA, et al.: Histopathologic risk factors in retinoblastoma in India. Arch Pathol Lab Med 133 (8): 1210-4, 2009.  [PUBMED Abstract]

  6. Sastre X, Chantada GL, Doz F, et al.: Proceedings of the consensus meetings from the International Retinoblastoma Staging Working Group on the pathology guidelines for the examination of enucleated eyes and evaluation of prognostic risk factors in retinoblastoma. Arch Pathol Lab Med 133 (8): 1199-202, 2009.  [PUBMED Abstract]

  7. Chantada GL, Dunkel IJ, Antoneli CB, et al.: Risk factors for extraocular relapse following enucleation after failure of chemoreduction in retinoblastoma. Pediatr Blood Cancer 49 (3): 256-60, 2007.  [PUBMED Abstract]

  8. Moscinski LC, Pendergrass TW, Weiss A, et al.: Recommendations for the use of routine bone marrow aspiration and lumbar punctures in the follow-up of patients with retinoblastoma. J Pediatr Hematol Oncol 18 (2): 130-4, 1996.  [PUBMED Abstract]

  9. Pratt CB, Meyer D, Chenaille P, et al.: The use of bone marrow aspirations and lumbar punctures at the time of diagnosis of retinoblastoma. J Clin Oncol 7 (1): 140-3, 1989.  [PUBMED Abstract]

  10. Zacharoulis S, Abramson DH, Dunkel IJ: More aggressive bone marrow screening in retinoblastoma patients is not indicated: the memorial Sloan-Kettering cancer center experience. Pediatr Blood Cancer 46 (1): 56-61, 2006.  [PUBMED Abstract]

  11. Abramson DH, Beaverson K, Sangani P, et al.: Screening for retinoblastoma: presenting signs as prognosticators of patient and ocular survival. Pediatrics 112 (6 Pt 1): 1248-55, 2003.  [PUBMED Abstract]

  12. Shields CL, Mashayekhi A, Demirci H, et al.: Practical approach to management of retinoblastoma. Arch Ophthalmol 122 (5): 729-35, 2004.  [PUBMED Abstract]

  13. Broaddus E, Topham A, Singh AD: Survival with retinoblastoma in the USA: 1975-2004. Br J Ophthalmol 93 (1): 24-7, 2009.  [PUBMED Abstract]