Abstract
Purpose
To assess the incidence, types, and outcomes of second primary tumors (SPT) in cases of retinoblastoma (RB) from a referral Tertiary eye care center
Methods
Retrospective chart review of 7 cases
Results
All 7 (100%) cases had bilateral RB at presentation. The mean age at diagnosis of RB was 16 months (median 7 months; range 5–72 months). Treatment of RB with intravenous chemotherapy was noted in 3 (43%) patients, 1 (14%) patient had received external beam radiotherapy (EBRT) to the orbit, 1 (14%) patient had received a combination of chemotherapy and orbital EBRT, while 4 (57%) patients had undergone primary enucleation of the worse eye and focal treatment of the better eye. The mean age at detection of SPT was 15 years (median 8 years; range 6–46 years). The mean time interval between diagnosis of RB and SPT was 13 years (median 7 years; range 1–41 years). The SPT’s included osteosarcoma of long bone (n = 2), eyelid sebaceous gland carcinoma (n = 2), ventricular ependymoma (n = 1), orbital neuroblastoma (n = 1), and acute lymphoblastic leukemia (n = 1). All patients received treatment for the SPT with either surgical excision (n = 2), intravenous chemotherapy (n = 1), or a combination of surgery/chemotherapy/radiotherapy (n = 4). Over a mean follow-up period of 8 years (median 8 years; range 4–11 years), one (14%) patient died, while other 6 (86%) patients are alive and well.
Conclusion
Though the incidence of SPT’s in cases of RB is rare, life-long follow-up is mandatory in at-risk patients.
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References
Kaliki S, Patel A, Iram S, Ramappa G, Mohamed A, Palkonda VAR (2019) Retinoblastoma in India: Clinical presentation and outcome in 1,457 patients (2,074 eyes). Retina 39(2):379–391
Broaddus E, Topham A, Singh AD (2009) Survival with retinoblastoma in the USA: 1975–2004. Br J Ophthalmol 93(1):24–27
MacCarthy A, Birch JM, Draper GJ et al (2009) Retinoblastoma: treatment and survival in Great Britain 1963 to 2002. Br J Ophthalmol 93(1):38–39
Dommering CJ, Marees T, van der Hout AH et al (2012) RB1 mutations and second primary malignancies after hereditary retinoblastoma. Fam Cancer 11(2):225–233
Woo KI, Harbour JW (2010) Review of 676 second primary tumors in patients with retinoblastoma: association between age at onset and tumor type. Arch Ophthalmol 128(7):865–870
Laiginhas R, Falcão M (2019) 40 years of retinoblastoma: Better survival rates despite stable second primary neoplasms. J Pediatr Ophthalmol Strabismus 56(2):131
Chaussade A, Millot G, Wells C et al (2019) Correlation between RB1germline mutations and second primary malignancies in hereditary retinoblastoma patients treated with external beam radiotherapy. Eur J Med Genet 62(3):217–223
Temming P, Arendt M, Viehmann A et al (2017) Incidence of second cancers after radiotherapy and systemic chemotherapy in heritable retinoblastoma survivors: A report from the German reference center. Pediatr Blood Cancer 64(1):71–80
Jiménez I, Laé M, Tanguy M-L et al (2020) Craniofacial second primary tumors in patients with germline retinoblastoma previously treated with external beam radiotherapy: A retrospective institutional analysis. Pediatr Blood Cancer 67(4):e28158
Fernandes AG, Pollock BD, Rabito FA (2018) Retinoblastoma in the United States: a 40-year incidence and survival analysis. J Pediatr Ophthalmol Strabismus 55(3):182–188
Shields CL, Mashayekhi A, Au AK et al (2006) The international classification of Retinoblastoma predicts chemoreduction success. Ophthalmology 113(12):2276–2280
Chantada G, Doz F, Antoneli CB et al (2006) A proposal for an international retinoblastoma staging system. Pediatr Blood Cancer 47(6):801–805
Kleinerman RA, Tucker MA, Tarone RE et al (2005) Risk of new cancers after radiotherapy in long-term survivors of retinoblastoma: an extended follow-up. J Clin Oncol 23(10):2272–2279
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
MacCarthy A, Bayne AM, Draper GJ et al (2009) Non-ocular tumours following retinoblastoma in Great Britain 1951 to 2004. Br J Ophthalmol 93(9):1159–1162
Turaka K, Shields CL, Meadows AT, Leahey A (2012) Second malignant neoplasms following chemoreduction with carboplatin, etoposide, and vincristine in 245 patients with intraocular retinoblastoma. Pediatr Blood Cancer 59(1):121–125
Marees T, Moll AC, Imhof SM, de Boer MR, Ringens PJ, van Leeuwen FE (2008) Risk of second malignancies in survivors of retinoblastoma: more than 40 years of follow-up. J Natl Cancer Inst 100(24):1771–1779
Abramson DH, Melson MR, Dunkel IJ, Frank CM (2001) Third (fourth and fifth) nonocular tumors in survivors of retinoblastoma. Ophthalmology 108(10):1868–1876
Yu CL, Tucker MA, Abramson DH et al (2009) Cause-specific mortality in long-term survivors of retinoblastoma. J Natl Cancer Inst 101(8):581–591
Sheen V, Tucker MA, Abramson DH, Seddon JM, Kleinerman RA (2008) Cancer screening practices of adult survivors of retinoblastoma at risk of second cancers. Cancer 113(2):434–441
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Support provided by The Operation Eyesight Universal Institute for Eye Cancer (SK) and Hyderabad Eye Research Foundation (SK), Hyderabad, India. The funders had no role in the preparation, review or approval of the manuscript.
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Kaliki, S., Palkonda, V.A.R. Second primary tumors in retinoblastoma survivors: a study of 7 Asian Indian patients. Int Ophthalmol 40, 3303–3308 (2020). https://doi.org/10.1007/s10792-020-01517-y
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DOI: https://doi.org/10.1007/s10792-020-01517-y