Abstract
Retinoblastoma can extend outward through different structures of the eye. It may disseminate through the optic nerve into the CNS and through the sclera to the orbit. Retinoblastoma can also give rise to systemic metastasis after gaining access to the choroidal circulation or after locoregional dissemination to the orbit and lymph nodes. Usual metastatic sites of retinoblastoma include the CNS, bone and the bone marrow (MacKay et al. 1984). Less frequently, retinoblastoma can metastasize to other organs, such as the liver or distant lymph nodes.
The presenting signs and symptoms of metastatic retinoblastoma are quite variable and depend on the site or sites of involvement. In patients who have previously undergone enucleation, orbital recurrences often present with the parental observation that the prosthesis is no longer fitting well. More extensive orbital disease may present as a visible mass. Central nervous system disease can occur as optic nerve disease tracking posteriorly into the brain, or as diffuse leptomeningeal involvement. Again, signs and symptoms are variable, depending on the locations involved and the degree of involvement, but may include headache, irritability, emesis and/or focal neurological signs. Bone disease may present with pain, and bone marrow disease may present with abnormally low blood counts, but often, disease at those sites and liver disease may be asymptomatic and discovered only during evaluation of the extent of the disease(Table 8.1).
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Abiose A (1979) Pattern of retinal diseases in Lagos. Ann Ophthalmol 11(7):1067-1072
Abramson DH (2005) Retinoblastoma in the 20th century: past success and future challenges the Weisenfeld lecture. Invest Ophthalmol Vis Sci 46(8):2683-2691
Abramson DH, Andracchi S (1997) Orbital avitene granuloma formation after enucleation for intraocular retinoblastoma. Am J Ophthalmol 123(4):567-569
Abramson DH, Ellsworth RM (1980) The surgical management of retinoblastoma. Ophthalmic Surg 11(9):596-598
Abramson DH, Schefler AC, Almeida D et al (2003a) Optic nerve tissue shrinkage during pathologic processing after enucleation for retinoblastoma. Arch Ophthalmol 121(1):73-75
Abramson DH, Beaverson K, Sangani P et al (2003b) Screening for retinoblastoma: presenting signs as prognosticators of patient and ocular survival. Pediatrics 112(6 Pt 1):1248-1255
Abramson DH, Beaverson KL, Chang ST et al (2004) Outcome following initial external beam radiotherapy in patients with Reese-Ellsworth group Vb retinoblastoma. Arch Ophthalmol 122(9):1316-1323
Antoneli CB, Steinhorst F, de Cassia Braga Ribeiro K et al (2003) Extraocular retinoblastoma: a 13-year experience. Cancer 98(6):1292-1298
Bellaton E, Bertozzi AI, Behar C et al (2003) Neoadjuvant chemotherapy for extensive unilateral retinoblastoma. Br J Ophthalmol 87(3):327-329
Bowman RJ, Mafwiri M, Luthert P et al (2006) Outcome of retinoblastoma in east Africa. Pediatr Blood Cancer 50(1):160-162
Chantada GL, de Davila MT, Fandino A et al (1999a) Retinoblastoma with low risk for extraocular relapse. Ophthalmic Genet 20(3):133-140
Chantada G, Fandino A, Manzitti J et al (1999b) Late diagnosis of retinoblastoma in a developing country. Arch Dis Child 80(2):171-174
Chantada G, Fandino A, Casak S et al (2003) Treatment of overt extraocular retinoblastoma. Med Pediatr Oncol 40(3):158-161
Chantada GL, Dunkel IJ, de Davila MT et al (2004a) Retinoblastoma patients with high risk ocular pathological features: who needs adjuvant therapy? Br J Ophthalmol 88(8):1069-1073
Chantada G, Fandino A, Davila MT et al (2004b) Results of a prospective study for the treatment of retinoblastoma. Cancer 100(4):834-842
Chantada G, Doz F, Antoneli CB et al (2006a) A proposal for an international retinoblastoma staging system. Pediatr Blood Cancer 47(6):801-805
Chantada GL, Rossi J, Casco F et al (2006b) An aggressive bone marrow evaluation including immunocytology with GD2 for advanced retinoblastoma. J Pediatr Hematol Oncol 28(6):369-373
Chantada GL, Casco F, Fandino AC et al (2007a) Outcome of patients with retinoblastoma and postlaminar optic nerve invasion. Ophthalmology 114(11):2083-2089
Chantada GL, Dunkel IJ, Antoneli CB et al (2007b) Risk factors for extraocular relapse following enucleation after failure of chemoreduction in retinoblastoma. Pediatr Blood Cancer 49(3):256-260
Chantada GL, Doz F, Orjuela M, Qaddoumi I, Sitorus R, Kepak T, Furmanchuk A, Castellanos M, Sharma T, Chevez-Barrios P, Rodriguez-Galindo C, on behalf of the International Retinoblastoma Staging Working Group (2008) World disparities in risk definition and management of retinoblastoma. A report from the International Retinoblastoma Staging Working Group. Pediatr Blood Cancer 50(3):692-694
de Graaf P, Moll AC, Imhof SM et al (2006) Retinoblastoma and optic nerve enhancement on MRI: not always extraocular tumour extension. Br J Ophthalmol 90(6):800-801
Doz F, Khelfaoui F, Mosseri V et al (1994) The role of chemotherapy in orbital involvement of retinoblastoma. The experience of a single institution with 33 patients. Cancer 74(2):722-732
Finger PT, Harbour JW, Karcioglu ZA (2002) Risk factors for metastasis in retinoblastoma. Surv Ophthalmol 47(1):1-16
Grabowski EF, Abramson DH (1987) Intraocular and extraocular retinoblastoma. Hematol Oncol Clin North Am 1(4):721-735
Gunduz K, Muftuoglu O, Gunalp I et al (2006) Metastatic retinoblastoma clinical features, treatment, and prognosis. Ophthalmology 113(9):1558-1566
Honavar SG, Singh AD, Shields CL et al (2002) Postenucleation adjuvant therapy in high-risk retinoblastoma. Arch Ophthalmol 120(7):923-931
Howarth C, Meyer D, Hustu HO et al (1980) Stage-related combined modality treatment of retinoblastoma. Results of a prospective study. Cancer 45(5):851-858
Hungerford J, Kingston J, Plowman N (1987) Orbital recurrence of retinoblastoma. Ophthalmic Paediatr Genet 8(1):63-68
Khelfaoui F, Validire P, Auperin A et al (1996) Histopathologic risk factors in retinoblastoma: a retrospective study of 172 patients treated in a single institution. Cancer 77(6):1206-1213
Leal-Leal C, Flores-Rojo M, Medina-Sanson A et al (2004) A multicentre report from the Mexican Retinoblastoma Group. Br J Ophthalmol 88(8):1074-1077
Leal-Leal CA, Rivera-Luna R, Flores-Rojo M et al (2006) Survival in extra-orbital metastatic retinoblastoma: treatment results. Clin Transl Oncol 8(1):39-44
MacKay CJ, Abramson DH, Ellsworth RM (1984) Metastatic patterns of retinoblastoma. Arch Ophthalmol 102(3):391-396
Magramm I, Abramson DH, Ellsworth RM (1989) Optic nerve involvement in retinoblastoma. Ophthalmology 96(2):217-222
(1992) National registry of retinoblastoma in Japan (1975-1982). The Committee for the National Registry of Retinoblastoma. Nippon Ganka Gakkai Zasshi 96(11):1433-1442
Ozkan A, Pazarli H, Celkan T et al (2006) Retinoblastoma in Turkey: survival and clinical characteristics 1981-2004. Pediatr Int 48(4):369-373
Rodriguez-Galindo C, Wilson MW, Haik BG et al (2003)Treatment of metastatic retinoblastoma. Ophthalmology 110:1237-1240
Rubin CM, Robison LL, Cameron JD et al (1985) Intraocular retinoblastoma group V: an analysis of prognostic factors. J Clin Oncol 3(5):680-685
Schvartzman E, Chantada G, Fandino A et al (1996) Results of a stage-based protocol for the treatment of retinoblastoma. J Clin Oncol 14(5):1532-1536
Shields CL, Shields JA, Baez KA et al (1993) Choroidal invasion of retinoblastoma: metastatic potential and clinical risk factors. Br J Ophthalmol 77(9):544-548
Shields CL, Shields JA, Baez K et al (1994) Optic nerve invasion of retinoblastoma. Metastatic potential and clinical risk factors. Cancer 73(3):692-698
Shields CL, Honavar S, Shields JA et al (2000) Vitrectomy in eyes with unsuspected retinoblastoma. Ophthalmology 107(12):2250-2255
Stannard CE, Sealy R, Sevel D et al (1975) Treatment of malignant meningitis in retinoblastoma. Br J Ophthalmol 59(7):362-365
Stannard C, Lipper S, Sealy R et al (1979) Retinoblastoma: correlation of invasion of the optic nerve and choroid with prognosis and metastases. Br J Ophthalmol 63(8):560-570
Stannard C, Sealy R, Hering E et al (2002) Postenucleation orbits in retinoblastoma: treatment with 125I brachytherapy. Int J Radiat Oncol Biol Phys 54(5):1446-1454
(1992) Survival rate and risk factors for patients with retinoblastoma in Japan. The Committee for the National Registry of Retinoblastoma. Jpn J Ophthalmol 36(2):121-131
Uusitalo MS, Van Quill KR, Scott IU et al (2001) Evaluation of chemoprophylaxis in patients with unilateral retinoblastoma with high-risk features on histopathologic examination. Arch Ophthalmol 119(1):41-48
Yamane S, Shirai C, Arimoto A et al (1999) Disseminated retinoblastoma successfully treated with myeloablative chemotherapy - implication for molecular detection of minimal residual disease. Bone Marrow Transplant 23(9):971-974
Zelter M, Gonzalez G, Schwartz L et al (1988) Treatment of retinoblastoma. Results obtained from a prospective study of 51 patients. Cancer 61(1):153-160
Zelter M, Damel A, Gonzalez G et al (1991) A prospective study on the treatment of retinoblastoma in 72 patients. Cancer 68(8):1685-1690
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2010 Springer Science+Business Media, LLC
About this chapter
Cite this chapter
Chantada, G.L., Dunkel, I.J. (2010). Treatment of Extraocular and Metastatic Retinoblastoma. In: Rodriguez-Galindo, C., Wilson, M. (eds) Retinoblastoma. Pediatric Oncology. Springer, Boston, MA. https://doi.org/10.1007/978-0-387-89072-2_8
Download citation
DOI: https://doi.org/10.1007/978-0-387-89072-2_8
Published:
Publisher Name: Springer, Boston, MA
Print ISBN: 978-0-387-89071-5
Online ISBN: 978-0-387-89072-2
eBook Packages: MedicineMedicine (R0)