Retinoblastoma natural history, complications, and prognosis

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Overview

If left untreated, the tumor fills the eye and completely destroys the globe in six months .Retinoblastoma is a rapidly growing tumor. Metastatic spread begins after six months and metastasized tumor is very rare at presentation. The tumor may spread through the subarachnoid space to the contralateral optic nerve or through the cerebrospinal fluid to the central nervous system or hematogenously to the lung, bone, or brain or by lymphatics if the tumor spreads anteriorly into the conjunctivae and eyelids, or extends into extraocular tissue. The most common routes of metastatic spread are direct infiltration via the optic nerve to the central nervous system, or spread via the choroid to the orbit. If untreated, death usually occurs in few years.

Natural History

If left untreated, retinoblastoma may progress to develop seeding in the eye, leading to retinal detachment, necrosis and invasion of the orbit, optic nerve invasion, and central nervous system invasion. Generally, metastasis may begin within 4 months of diagnosis. Metastasis usually occur through direct invasion of the central nervous system via the optic nerve. Retinoblastoma may spread through the subarachnoid space to the contralateral optic nerve or through the cerebrospinal fluid to the central nervous system and may spread hematogenously to the lungs, bone, and brain.[1] The majority of untreated patients die of intracranial extension and disseminated disease within two one year. Spontaneous regression of the tumor is a rare occurrence but may occur in a small number of cases.[2][3][4]

Prognosis

  • Prognosis is generally good, and the survival rate of patients with retinoblastoma with treatment is approximately 95%, in the United States.[5] However, the prognosis for eye salvage is far less and usually depends on the stage of retinoblastoma at diagnosis.
  • In children with retinoblastoma that has invaded into the choroid, optic nerve, sclera, orbit or anterior chamber require chemotherapy as they are at greatest risk for metastasis and death. Those children without evidence of invasion do not require chemotherapy.
  • The goal of treatment in retinoblastoma is to preserve life and to prevent the loss of an eye, blindness, and other serious effects of treatment that reduce the patient's life span or the quality of life. With improvements in the diagnosis and management of retinoblastoma over the past several decades, metastatic retinoblastoma is observed less frequently in the United States and other developed nations.
  • As a result, other causes of retinoblastoma-related mortality in the first and subsequent decades of life, such as trilateral retinoblastoma and subsequent neoplasms (SNs), have become significant contributors to retinoblastoma-related mortality. Death from a subsequent neoplasms is the most common cause of death and contributes to more than 50% of deaths for patients with bilateral disease. In the United States, before the advent of chemoreduction as a means of treating heritable or bilateral disease, trilateral retinoblastoma contributed to more than 50% of retinoblastoma-related mortality in the first decade after diagnosis.

The features associated with poor prognosis in retinoblastoma include:[6]

  • Cataract
  • Delay in diagnosis of more than six months[7][8][9][10]
  • History of intraocular surgery, which may inadvertently contribute to vitreous seeding or, more importantly, to extraocular spread.[11][12]
  • Use of external beam radiotherapy, which contributes to the subsequent development of secondary cancers, particularly in patients with the heritable form of the disease[13][14][15]
  • Choroidal, optic nerve, scleral, or orbital invasion, which increases the risk of metastatic disease.[16][7][17][18][19]
  • Tumor anaplasia[19]
  • Larger tumor
  • Greater age
  • Evidence of optic nerve involvement


Complications

The complications of retinoblastoma may be

  • Local spread of tumor
  • Development of other tumors
  • Cataract from radiotherapy
  • Failure of tooth eruption after radiotherapy
  • Bony deformities

References

  1. Singh, Arun D., Carol L. Shields, and Jerry A. Shields. "Prognostic factors in retinoblastoma." Journal of pediatric ophthalmology and strabismus 37.3 (2000): 134.
  2. Sanborn GE, Augsburger JJ, Shields JA (1982). "Spontaneous regression of bilateral retinoblastoma". Br J Ophthalmol. 66 (11): 685–90. PMC 1039901. PMID 7126513.
  3. Kao LY, Yang ML (2005). "Spontaneous regression of retinoblastoma in a Taiwan series". J Pediatr Ophthalmol Strabismus. 42 (4): 228–32. PMID 16121553.
  4. Khodadoust AA, Roozitalab HM, Smith RE, Green WR (1977). "Spontaneous regression of retinoblastoma". Surv Ophthalmol. 21 (6): 467–78. PMID 898013.
  5. Lin P, O'Brien JM (2009). "Frontiers in the management of retinoblastoma". Am J Ophthalmol. 148 (2): 192–8. doi:10.1016/j.ajo.2009.04.004. PMID 19477707.
  6. Singh AD, Shields CL, Shields JA (2000). "Prognostic factors in retinoblastoma". J Pediatr Ophthalmol Strabismus. 37 (3): 134–41, quiz 168-9. PMID 10845413.
  7. 7.0 7.1 Messmer EP, Heinrich T, Höpping W, de Sutter E, Havers W, Sauerwein W (1991). "Risk factors for metastases in patients with retinoblastoma". Ophthalmology. 98 (2): 136–41. PMID 2008269.
  8. Goddard AG, Kingston JE, Hungerford JL (1999). "Delay in diagnosis of retinoblastoma: risk factors and treatment outcome". Br J Ophthalmol. 83 (12): 1320–3. PMC 1722906. PMID 10574806.
  9. Erwenne CM, Franco EL (1989). "Age and lateness of referral as determinants of extra-ocular retinoblastoma". Ophthalmic Paediatr Genet. 10 (3): 179–84. PMID 2587030.
  10. Kaliki S, Srinivasan V, Gupta A, Mishra DK, Naik MN (2015). "Clinical features predictive of high-risk retinoblastoma in 403 Asian Indian patients: a case-control study". Ophthalmology. 122 (6): 1165–72. doi:10.1016/j.ophtha.2015.01.018. PMID 25841975.
  11. Stevenson KE, Hungerford J, Garner A (1989). "Local extraocular extension of retinoblastoma following intraocular surgery". Br J Ophthalmol. 73 (9): 739–42. PMC 1041868. PMID 2804029.
  12. Shields CL, Honavar S, Shields JA, Demirci H, Meadows AT (2000). "Vitrectomy in eyes with unsuspected retinoblastoma". Ophthalmology. 107 (12): 2250–5. PMID 11097606.
  13. Wong FL, Boice JD, Abramson DH, Tarone RE, Kleinerman RA, Stovall M; et al. (1997). "Cancer incidence after retinoblastoma. Radiation dose and sarcoma risk". JAMA. 278 (15): 1262–7. PMID 9333268.
  14. Abramson DH, Frank CM (1998). "Second nonocular tumors in survivors of bilateral retinoblastoma: a possible age effect on radiation-related risk". Ophthalmology. 105 (4): 573–9, discussion 579-80. doi:10.1016/S0161-6420(98)94006-4. PMID 9544627.
  15. Moll AC, Imhof SM, Bouter LM, Tan KE (1997). "Second primary tumors in patients with retinoblastoma. A review of the literature". Ophthalmic Genet. 18 (1): 27–34. PMID 9134547.
  16. Finger PT, Harbour JW, Karcioglu ZA (2002). "Risk factors for metastasis in retinoblastoma". Surv Ophthalmol. 47 (1): 1–16. PMID 11801265.
  17. Shields CL, Shields JA, Baez K, Cater JR, De Potter P (1994). "Optic nerve invasion of retinoblastoma. Metastatic potential and clinical risk factors". Cancer. 73 (3): 692–8. PMID 8299091.
  18. Shields CL, Shields JA, Baez KA, Cater J, De Potter PV (1993). "Choroidal invasion of retinoblastoma: metastatic potential and clinical risk factors". Br J Ophthalmol. 77 (9): 544–8. PMC 513947. PMID 8218048.
  19. 19.0 19.1 Mendoza PR, Specht CS, Hubbard GB, Wells JR, Lynn MJ, Zhang Q; et al. (2015). "Histopathologic grading of anaplasia in retinoblastoma". Am J Ophthalmol. 159 (4): 764–76. doi:10.1016/j.ajo.2014.12.014. PMC 4361305. PMID 25528954.

See also

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