Abstract
Introduction
Retinoblastoma (RB) is the most frequent malignant eye tumor in childhood. In developing countries advanced stages are common. The purpose of this paper is to present our 21-year clinical experience with metastatic extra ocular RB patients treated with 5 different chemotherapy schemas at a single Mexican Pediatric referral center.
Materials and methods
A retrospective analysis was carried out reviewing the clinical characteristics of patients with metastatic RB. The information analyzed included the delay in diagnosis after first symptoms, age, sex, ocular staging, and anatomic site of metastases, treatment scheme, initial response and status at the last contact or date of death.
Results
Eighty-one patients were included; age range was from 3 to 80 months. The most common site of metastasis was central nervous system (83.9%). From those patients treated with chemotherapy (n=74), 89.2% presented a complete initial response (n=66). Early mortality occurred in 7 cases before any treatment. Fifty-six received treatment and died with progressive disease. All patients without radiotherapy died with tumor activity (n=15). The use of cisplatin was related with longer disease free intervals; no other variable was related with survival. Four patients were alive and disease free at 33 to 144.3 months of follow up from diagnosis. The prevalent cause of death was tumor progression.
Conclusions
In our experience, metastatic RB has a very high mortality rate in spite of the use of different chemotherapy regimens.
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References
Grabowski EF, Abramson DH, Intraocular and extraocular retinoblastoma. Hematol Oncol Clin North Am. 1987;1(4):721–35.
Gatta G, Capocaccia R, Stiller C, Kaatsch P, Berrino F, Terenziani M. Childhood cancer survival trends in Europe: a EU-ROCARE Working Group study. J Clin Oncol. 2005;23(16):3742–51.
Wessels G, Hesseling PB. Incidence and frequency rates of childhood cancer in Namibia. S Afr Med J. 1997;87(7):885–9.
Schvartzman E, Chantada G, Fandino A, de Davila MT, Raslawski E, Manzitti J. Results of a stage-based protocol for the treatment of retinoblastoma. J Clin Oncol. 1996;14(5):1532–6.
Messmer EP, Heinrich T, Hopping W, de Sutter E, Havers W, Sauerwein W. Risk factors for metastases in patients with retionoblastoma. Ophthalmology. 1991;98(2):136–41.
Ebata K, Mizutani H, Kaneda T, Horibe K. Metastatic retinoblastoma to the orofacial region. J Oral Maxillofac Surg. 1991;49(10):1120–3.
Moshfeghi DM, Wilson MW, Haik BG, Hill DA, Rodriguez-Galindo C, Pratt CB. Retinoblastoma metastatic to the ovary in a patient with Waardenburg syndrome. Am J Ophthalmol. 2002;133(5):716–8.
Zubizarreta P, Chantada G: Circulating retinoblastoma cells in a patient with metastatic disease. Ophthalmic Genet. 1999; 20(3):189–91.
Doz F, Neuenschwander S, Plantaz D, et al. Etoposide and carboplatin in extraocular retinoblastoma: a study by the Societe Francaise d'Oncologie Pediatrique. J Clin Oncol. 1995;13(4):902–9.
Saarinen UM, Sariola H, Hovi L. Recurrent disseminated retinoblastoma treated by high-dose chemotherapy, total body irradiation, and autologous bone marrow rescue. Am J Pediatr Hematol Oncol. 1991;13(3):315–9.
Michon J, Schleiermacher G. Autologous hematopoietic stem cell transplantation for pediatric solid tumors. Baillieres Best Pract Res Clin Haematol. 1999;12(1–2):247–59.
Rodríguez-Galindo C, Wilson MW, Haik BG, et al. Treatment of metastatic retinoblastoma. Ophthalmology. 2005;110(6):1237–40.
Pratt C.B. Management of malignant solid tumors in children. Pediatr Clin North Am. 1972;19(4):1141–55.
Reese AB, Ellsworth RM. Management of retinoblastoma. Ann N Y Acad Sci. 1964;114(2):958–62.
Chantada G, Fandino A, Manzitti J, Urrutia L, Schvartzman E. Late diagnosis of retinoblastoma in a developing country. Arch Dis Child. 1990;80(2):171–4.
Finger PT, Czechonska G, Demirci H, Rausen A. Chemotherapy for retinoblastoma: a current topic. Drugs. 1999;58(6):985–96.
Doz F, Khelfaoui F, Mosseri V, et al. The role of chemotherapy in orbital involvement of retinoblastoma. The experience of a single institution with 33 patients. Cancer. 1994;74(2):722–52.
Chantada G, Fandino A, Gasak S, Manzitti J, Raslawski E, Schvartzman E. Treatment of overt extraocular retinoblastoma. Med Pediatr Oncol. 2003;40(3):158–61.
Saleh RA, Gross S, Cassano W, Gee A. Metastatic retinoblastoma successfully treated with immunomagnetic purged autologous bone marrow transplantation. Cancer. 1988;62(11):2301–3.
Frangoul H, Ames MM, Mosher RB, et al. Phase 1 study of topotecan administered as a 21-day continous, infusion in children with recurrent solid tumors: a report from the Children's Cancer Group. Clin Cancer Res. 1999;5(12):5956–62.
Doz F, Gentet JC, Pein F, et al. Phase 1 trial and pharmacological study of a 3-hour paclitaxel infusion in children with refractory solid tumours: a SFOP study. Br J Cancer. 2001;84(5):604–10.
Chantada GL, Fandino A, Mato G, Casak S. Phase II window of idarubicin in children with extraocular retinoblastoma. J Clin Oncol. 1999;17(6):1847–50.
Gururangan S, Friedman HS, Phillips PC, et al. Phase I study of intrathecal (it) Spartaject busulfan (Bu) in children with neoplastic emningitis (NM): A Pediatric Brain Tumour Consortium Trial [abstract]. Proc Am Soc Clin Oncol. 2003;22:456.
Tacyildiz N, Yavuz G, Unal E, Gunduz K, Gunalp I, Ekinci C. Encouraging result of tamoxifen in a retinoblastoma patient with central nervous system metastasis. Pediatr Hematol Oncol. 2003;20(6):473–6.
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Leal-Leal, C.A., Rivera-Luna, R., Flores-Rojo, M. et al. Survival in extra-orbital metastatic retinoblastoma: treatment results. Clin Transl Oncol 8, 39–44 (2006). https://doi.org/10.1007/s12094-006-0093-x
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DOI: https://doi.org/10.1007/s12094-006-0093-x