Retinoblastoma – pattern, presentation and management: a quintessential experience of 5 years

Authors

  • Anvesh Karthik Yalavarthy Department of Medical Oncology, Vydehi Institute of Medical Sciences and Research Centre, Bangalore, Karnataka, India
  • Manjunath I. Nandennavar Department of Medical Oncology, Vydehi Institute of Medical Sciences and Research Centre, Bangalore, Karnataka, India
  • Shashidhar V. Karpurmath Department of Medical Oncology, Vydehi Institute of Medical Sciences and Research Centre, Bangalore, Karnataka, India

DOI:

https://doi.org/10.18203/2320-6012.ijrms20164028

Keywords:

Chemotherapy, Developing countries, Enucleation, Survival, Retinoblastoma

Abstract

Background: Retinoblastoma (RB) is the most common intraocular malignancy of childhood. It accounts for 10-15% of cancers that occur in infants. Objective of the study was to determine the pattern, presentation and management of Retinoblastoma (RB) patients at a tertiary cancer care center in South India, during a period of 5 years (1st November 2009 to 1st December 2014).

Methods: This study was a retrospective analysis of the medical records of patients diagnosed as having retinoblastoma obtained from hospital information system and our cancer registry. Data sought was demographic characteristics, clinical presentation, investigations done, the methods of management and the treatment outcome of retinoblastoma patients.

Results: Total number of cases studied was 31 and total number of eyes studied was 47. Out of 31 cases, 58% were female. 52% had bilateral involvement. The median age at presentation was 24 months. The commonest mode of presentation was leukocoria (55%) followed by proptosis (22%). Out of 47 eyes studied, 87% were advanced tumours belonging to Group D or Group E. Metastasis to the central nervous system was noted in 22.6% patients belonging to either Group D or Group E. Out of the 31 patients, 64.6% patients underwent enucleation. 6 out of 31 cases succumbed to death.

Conclusions: Retinoblastoma continues to be a challenge in developing countries. Lack of awareness and inaccessibility to proper healthcare facilities are major stumbling blocks in achieving high cure rates. Educating the public and healthcare professionals, importance of early diagnosis and prompt referral are vital in reducing morbidity and mortality associated with the disease.

References

Knudson AG Jr. Mutation and cancer: statistical study of retinoblastoma. Proc Natl Acad Sci USA. 1971;68:820-3.

Shields JA, Shields CL. Management and prognosis of retinoblastoma. In: Intraocular Tumors: A Text and Atlas. Philadelphia, Pa: WB Saunders. 1992.

Shields JA, Shields CL. Retinoblastoma. In: Shields JA, Shields CL, eds. Atlas of Intraocular Tumors. Philadelphia, Pa: Lippincott Williams & Wilkins. 1999.

Howlader N, Noone AM, Krapcho M, Garshell J, Miller D, Altekruse SF, et al. SEER Cancer Statistics Review, 1975-2012, National Cancer Institute. Bethesda, MD. Available at: http://seer.cancer.gov/ csr/1975_2012

Arora RS, Eden T, Kapoor G. Epidemiology of childhood cancer in India. Indian J Cancer. 2009;46:264-73.

Swaminathan R, Rama R, Shanta V. Childhood cancers in Chennai, India, 1990-2001: incidence and survival. Int J Cancer. 2008;122:2607-11.

Tyagi BB, Manoharan N, Raina V. Childhood Cancer Incidence in Delhi, 1996-2000. Indian J Med Paediatr Oncol. 2006;27:13-8.

L Satyanarayana, Asthana S, Labani P. Childhood Cancer Incidence in India: A Review of Population-Based Cancer Registries. Indian Pediatr. 2014;51:218-20.

Sant M, Capocaccia R, Badioni V. Eurocare working group. Survival for retinoblastoma in Europe. Eur J Cancer. 2001;37:730-5.

Broaddus E, Topham A, Singh AD. Survival with retinoblastoma in the USA: 1975–2004. Br J Ophthalmol. 2009;93:24-7.

Canturk S, Qaddoumi I, Khetan V. Survival of retinoblastoma in less-developed countries impact of socioeconomic and health-related indicators. Br J Ophthalmol. 2010;94:1432-6.

Bowman RJ, Mafwiri M, Luthert P, Luande J and Wood M. Outcome of retinoblastoma in East Africa. Pediatr Blood Cancer. 2008;50:160-2.

Kao LY, Su WW, Lin YW. Retinoblastoma in Taiwan: Survival and clinical characteristics 1978–2000. Jpn J Ophthalmol. 2002;46:577-80.

Nyawira G, Kahaki K, Kariuki-Wanyoike M. Survival among retinoblastoma patients at Kenyatta National Hospital, Kenya. J Ophthalmol East Cent S Afr. 2013;17:15-9.

Chawla B, Hasan F, Azad R, Seth R, Upadhyay AD, Pathy S et al. Clinical presentation and survival of retinoblastoma in Indian children. Br J Ophthalmol. 2015;10:1-7.

Murphree AL, Munier FL. Retinoblastoma. In: Ryan SJ, ed. Retina, 2nd ed. St Louis: Mosby, 1994;605-6.

Linn Murphee A. Intraocular retinoblastoma: The case for a new group classification. Opthalmolm Clin North Am. 2005;18:41-53.

Shields CL, MeadowsAT, Leahey AM, Shields JA. Continuing challenges in the management of retinoblastoma with chemotherapy. Retina. 2004;24(6):849-62.

Friedman DL, Himelstein B, Shields CL, Shields JA, Needle M, Miller D et al. Chemo-reduction and local ophthalmic therapy for intraocular retinoblastoma. J ClinOncol. 2000;18:12-7.

Naseripour M, Nazari H, Bakhtiari P, Modarres-zadeh M, Vosough P, Ausari M, et al. Retinoblastoma in Iran: outcomes in terms of patient survival and globe survival. Br J Ophthalmol. 2009;93:28-32.

Chang CY, Chiou TJ, Hwang B, Bai LY, Hsu WM, Hsieh YL. Retinoblastoma in Taiwan: survival rate and prognostic factors. Jpn J Ophthalmol. 2006;50:242-9.

Essuman V, Ntim-Amponsah CT, Akafo S, Renner L, Edusei L. Presentation of retinoblastoma at a paediatric eye clinic in Ghana. Ghana Med J. 2010;44:10-5.

Berman EL, Donaldson CE, Giblin M, Martin FJ. Outcomes in retinoblastoma, 1974–2005: The Children’s Hospital, Westmead. Clin Experiment Ophthalmol. 2007;35:5-12.

Butros LJ, Abramson DH, Dunkel IJ. Delayed diagnosis of retinoblastoma: analysis of degree, cause, and potential sequences. Pediatrics. 2002;109:e45.

Moll AC, Kuik DJ, Bouter LM, Den Otter W, Bezemer PD, Koten JW, et al. Incidence and survival of retinoblastoma in The Netherlands: a register based study 1862-1995. Br J Ophthalmol. 1997;81:559-62.

Kiss S, Leiderman YI, Mukai S. Diagnosis, classification, and treatment of retinoblastoma. Int Ophthalmol Clin. 2008;48:135-47.

Abramson DH, Frank CM, Susman M, Whalen MP, Dunkel IJ, Boyd NW III. Presenting signs of retinoblastoma. J Pediatr. 1998;132:505-8.

Abramson DH, Beaverson K, Sangani P, Vora RA, Lee TC, Hochberg HM, et al. Screening for retinoblastoma: presenting signs as prognosticators of patient and ocular survival. Pediatrics. 2003;112:1248-55.

Owoeye JF, Afolayan EA, Ademola-Popoola DS. Retinoblastoma—a clinic-pathological study in Ilorin, Nigeria. Afr J Health Sci. 2005;12:94-100.

Zhao J, Li S, Shi J, Wang N. Clinical presentation and group classification of newly diagnosed intraocular retinoblastoma in China. Br J Ophthalmol. 2011;95:1372-5.

MacCarthy A, Draper GJ, Steliarova-Foucher E, Kingston JE. Retinoblastoma incidence and survival in European children (1978–1997). Report from the Automated Childhood Cancer Information System project. Eur J Cancer. 2006;42:2092-102.

Sanders BM, Draper GJ, Kingston JE. Retinoblastoma in Great Britain 1969–80: incidence, treatment, and survival. Br J Ophthalmol. 1988;72:576-83.

Survival rate and risk factors for patients with retinoblastoma in Japan. The Committee for the National Registry of Retinoblastoma. Jpn J Ophthalmol. 1992;36:121-31.

Waddell KM, Kagame K, Ndamira A, Twinamasiko A, Picton SV, Simmons IG, et al. Clinical features and survival among children with retinoblastoma in Uganda. Br J Ophthalmol. 2015;99:387-90.

Shields CL, Shields JA, Needle M, de Potter P, Kheterpal S, Hamada A, et al. Combined chemoreduction and adjuvant treatment for intraocular retinoblastoma. Ophthalmology. 1997;104:2101-11.

Abramson DH, Frank CM. Second nonoculartumors in survivors of bilateral retinoblastoma: a possible age effect on radiation-related risk. Ophthalmology. 1998;105(4):573-80.

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Published

2016-12-16

How to Cite

Yalavarthy, A. K., Nandennavar, M. I., & Karpurmath, S. V. (2016). Retinoblastoma – pattern, presentation and management: a quintessential experience of 5 years. International Journal of Research in Medical Sciences, 4(12), 5115–5119. https://doi.org/10.18203/2320-6012.ijrms20164028

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Original Research Articles