XIN LUO, HUIJING YE, YUN-GANG DING, YI DU, HUASHENG YANG

Clinical characteristics and prognosis of patients with retinoblastoma: 8-year follow-up

Background/aim: The aim of this study was to investigate the potential correlation between clinical characteristics and prognosis of patients with retinoblastoma (Rb). Materials and methods: This retrospective study included 314 Rb patients. Clinical data including laterality of eyes, sex, age, presenting signs, lag time, and survival were recorded and analyzed. Results: Leukocoria is the most common clinical presentation of Rb. Patients with isolated leukocoria had shorter lag time and exhibited a high survival rate (85%, 5 years). Patients with strabismus and blurred vision, and who were older and had longer lag time, exhibited an excellent survival rate (100% and 92.3%, respectively, 5 years). Patients with exophthalmos had the longest lag time and the lowest survival rate (17.8%, 5 years). The 5-year survival rate of patients with a lag time of ≤6 months was 84.7%, which was significantly higher than that of patients with a lag time of >6 months (64.7%). Conclusion: Leukocoria, strabismus, and blurred vision are mild clinical manifestations of Rb that are associated with better disease prognosis, whereas exophthalmos is an indicator of poor prognosis. Long lag time is a risk factor for the survival of Rb, which can be avoided. Early detection and treatment can greatly improve the survival of Rb patients.

Anahtar Kelimeler:

Retinoblastoma, diagnosis, prognosis

Clinical characteristics and prognosis of patients with retinoblastoma: 8-year follow-up

Keywords:

Retinoblastoma, diagnosis, prognosis,

PDF

___

Parkin DM, Stiller CA, Draper GJ. International Incidence of Childhood Cancer. IARC Scientific Publications Number 87. Lyon, France: International Agency for Research on Cancer; 1988.
Parkin DM, Kramarova E, Draper GJ. International Incidence of Childhood Cancer Volume 2. IARC Scientific Publications Number 144. Lyon, France: International Agency for Research on Cancer; 1998.
Shields JA, Shields CL, Meadows AT. Chemoreduction in the management of retinoblastoma. Am J Ophthalmol 2005; 140: 505–506.
Chantada G, Fandiño A, Manzitti J, Urrutia L, Schvartzman E. Late diagnosis of retinoblastoma in a developing country. Arch Dis Child 1999; 80:171–174.
Rodriguez-Galindo C, Wilson M, Chantada G, Fu L, Qaddoumi I, Antoneli C, Leal-Leal C, Sharma T, Barnoya M, Epelman S et al. Retinoblastoma: one world, one vision. Pediatrics 2008; 122: e763–770.
Murphree AL. Intraocular retinoblastoma: the case for a new group classification. Ophthalmol Clin North Am 2005; 18: 41–53.
Chantada G, Doz F, Antoneli CB, Grundy R, Clare Stannard FF, Dunkel IJ, Grabowski E, Leal-Leal C, Rodríguez-Galindo C, Schvartzman E et al. A proposal for an international retinoblastoma staging system. Pediatr Blood Cancer 2006; 47: 801–805.
Gatta G, Capocaccia R, Coleman MP, Ries LA, Berrino F. Childhood cancer survival in Europe and the United States. Cancer 2002; 95: 1767–1772.
Chang CY, Ghiou TJ, Hwang B, Bai LY, Hsu WM, Hsieh YL. Retinoblastoma in Taiwan: survival rate and prognostic factors. Jpn J Ophthalmol 2006; 50: 242–249.
Naseripour M, Nazari H, Bakhtiari P, Modarres-Zadeh M, Vosough P, Ausari M. Retinoblastoma in Iran: outcomes in terms of patients’ survival and globe survival. Br J Ophthalmol 2009; 93: 28–32.
Chantada GL, Dunkel IJ, Qaddoumi I, Antoneli CB, Totah A, Canturk S, Nawaiseh I, Fandiño A, Pífano I, Peksayar G et al. Familial retinoblastoma in developing countries. Pediatr Blood Cancer 2009; 53: 338–342.
Bowman RJ, Mafwiri M, Luthert P, Luande J, Wood M. Outcome of retinoblastoma in east Africa. Pediatr Blood Cancer 2008; 50: 160–162.
Luna-Fineman S, Barnoya M, Bonilla M, Fu L, Baez F, Rodríguez-Galindo C. Retinoblastoma in Central America: Report from the Central American Association of Pediatric Hematology Oncology (AHOPCA). Pediatr Blood Cancer 2011; 58: 545–550.
Ozkan A, Pazarli H, Celkan T, Karaman S, Apak H, Kaner G, Uzel O, Yildiz I. Retinoblastoma in Turkey: survival and clinical characteristics 1981–2004. Pediatr Int 2006; 48: 369–373.
Ozdemir H, Tacyildiz N, Unal E, Yavuz G, Ugur H, Gunduz K. Clinical and epidemiological characteristics of retinoblastoma: correlation with prognosis in a Turkish pediatric oncology center. Pediatr Hematol Oncol 2007; 24: 221–231.
Augsburger JJ, Bornfeld N, Giblin M. Malignant intraocular tumors-retinoblastoma. In: Yanoff M, editor. Ophthalmology. 1st ed. St. Louis, MO, USA: Mosby International; 1999. pp. 921–928.
Abramson DH, Beaverson K, Sangani P, Vora RA, Lee TC, Hochberg HM, Kirszrot J, Ranjithan M. Screening for retinoblastoma: presenting signs as prognosticators of patient and ocular survival. Pediatrics 2003; 112: 1248–1255.
Butros LJ, Abramson DH, Dunkel IJ. Delayed diagnosis of retinoblastoma: analysis of degree, cause, and potential consequences. Pediatrics 2002; 109: e45.
Abramson DH, Frank CM, Susman M, Whalen MP, Dunkel IJ, Boyd NW 3rd. Presenting signs of retinoblastoma. J Pediatr 1998; 132: 505–508.
Navo E, Teplisky D, Albero R, Fandino AC, Demirdjian G, Chantada GL. Clinical presentation of retinoblastoma in a middle-income country. J Pediatr Hematol Oncol 2012; 34: e97–101.
Aguirre Neto JC, Antoneli CB, Ribeiro KB, Castilho MS, Novaes PE, Chojniak MM, Arias V. Retinoblastoma in children older than 5 years of age. Pediatr Blood Cancer 2007; 48: 292–295.
Abdu L, Malami S. Clinicopathological pattern and management of retinoblastoma in Kano, Nigeria. Ann Afr Med 2011; 10: 214–218.
Badthu B, Sah SP, Thakur SK, Dulal S, Kumar S, Sood A, Das H, Sah RP. Clinical presentation of retinoblastoma in Eastern Nepal. Clin Experiment Ophthalmol 2005; 33: 386–389.
Bhurgri Y, Muzaffar S, Ahmed R, Ahmed N, Bhurgri H, Usman A, Faridi N, Malik J, Kazi LA, Bashir I et al. Retinoblastoma in Karachi, Pakistan. Asian Pac J Cancer Prev 2004; 5: 159–163.
Erwenne CM, Franco EL. Age and lateness of referral as determinants of extra-ocular retinoblastoma. Ophthalmic Paediatr Genet 1989; 10: 179–184.
Mahoney DH. Retinoblastoma. In: McMillan JA, editor. Oski’s Pediatrics: Principles and Practice. New York, NY, USA: Lippincott Williams & Wilkins; 1999. pp. 1525–1527.
Leander C, Fu LC, Peña A, Howard SC, Rodriguez-Galindo C, Wilimas JA, Ribeiro RC, Haik B. Impact of an education program on late diagnosis of retinoblastoma in Honduras. Pediatr Blood Cancer 2007; 49: 817–819.