Retinoblastomun tedavi kararındaki zorluklar

Dokuz yaşında erkek hasta, sağ gözde bulanık görme ve sinek uçuşması yakınmalarıyla başvurduğu dış merkezde tek taraflı retinoblastom tanısı aldıktan ve ilk kür kemoterapisi uygulandıktan sonra merkezimize başvurmuştu. Ailede retinoblastom öyküsü pozitifti. Oftalmolojik incelemede görme keskinliği her iki gözde 10/10, bilateral ön segment muayenesi doğal, göz içi basınçları normal idi. Fundus incelemesinde; sağ gözde optik disk ve makula olağan, retina nazalinde vitreusa protrüze olan kitle ve inferiorunda birkaç odak, vitreusta yaygın tohumlanma görüldü. Sağ gözdeki retinoblastom “Reese-Ellsworth Grup Vb”, “Uluslararası Retinoblastom Sınıflaması Grup E” olarak değerlendirildi. Sol göz normal bulundu. Hastanın başka merkezde aldığı ilk kür kemoterapisinden sonra merkezimizde dört kür VEC (vinkristin, etoposid, karboplatin) kemoterapisi kemoredüksiyon amacıyla uygulandı. Kemoterapi ile sağ gözdeki kitlede ve vitreus tohumlanmasında gerileme olmadığı görüldü. Retinoblastomun lokal ilerleme ve sistemik metastaz yapma riski de göz önüne alınarak, ileri evreli, kemoterapiye yanıtsız retinoblastomu olan sağ göze, 10/10 görmesine karşın enükleasyon uygulandı. Lokal tedavi ile görme keskinliği ve globun korunması planlanan olguda, tümörde kemoredüksiyon rejimi ile tümör regresyonu sağlanamadığından enükleasyon uygulanmıştır.

Difficulties in decision of retinoblastoma treatment

A nine year-old boy was admitted with a history of ‘blurred vision and floaters’ in his right eye. He was first admitted to another hospital and diagnosed with retinoblastoma by fundoscopic and radiological examinations. After receiving one course of center. Family history was positive for retinoblastoma in his cousin. Our ophthalmologic exa mination revealed full visual acuity in both eyes, preserved bilateral anterior segment and normal intraocular pressure. Fundus examination revealed a normal optic disc and macula, a protured mass into vitreous cavity in the nasal part of retina and a few focus in inferior part, diffuse seeding in the vitreous in his right eye. His left eye was normal. He had advanced intraocular retinoblastoma in the right eye, consistent with Group Vb by Reese-Ellsworth classification, and Group E by International Retinoblastoma Classification according to the ophtalmological examination and imaging studies. He received five courses of chemoreduction regimen with carboplatinum, etoposide and vincristine. Tumor size showed no regression and vitreous seeding did not disappear. He eventually underwent enucleation of the right eye although the visual acuity was full, since he had advanced intraocular disease with no response to chemoreduction regimen. Although visual acuity and globe integrity were planned with local treatment, enucleation was performed because of no tumor regression with chemoreduction regimen.

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  • 1. Hurwitz RL, Shields CL, Shields JA, Chevez-Barrios P, Hurwitz MY, Chintagumpala MM. Retinoblastoma. In: Pizzo PA, Poplack DG (eds). Principles and Practice of Pediatric Oncology, 5th ed. Philadelphia: Lippincott Williams & Wilkin 2006.
  • 2. Reese AB and Ellsworth RM. The evaluation and current concept of retinoblastoma therapy, Trans Am Acad Ophthalmol Otolaryngol 1963; 67:164–172.
  • 3. Murphree AL. Intraocular retinoblastoma: the case for a new group classification. Ophthalmol Clin North Am 2005;18:41-53.
  • 4. Kutluk T, Yesilipek A. On behalf of the Turkish Pediatric Oncology Group (TPOG) and Turkish Pediatric Hematology Society (TPHD). Pediatric Tumor Registry for 2002- 2005 in Turkey. J Pediatr Hematol Oncol 2007; 29:12-13.
  • 5. Ozkan A, Pazarli H, Celkan T et al. Retinoblastoma in Turkey: survival and clinical characteristics 1981-2004. Pediatr Int 2006;48:369-373.
  • 6. 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.
  • 7. de Aguirre Neto JC, Antoneli CB, Ribeiro KB et al. Retinoblastoma in children older than 5 years of age. Pediatr Blood Cancer 2007;48:292-295.
  • 8. Shields CL, Honavar SG, Meadows AT et al. Chemoreduction plus focal therapy for retinoblastoma: factors predictive of need for treatment with external beam radiotherapy or enucleation. Am J Ophthalmol 2002; 133:657-664.
  • 9. Shields CL, Shields JA, Cater J, Othmane I, Singh AD, Micaily B. Plaque radiotherapy for retinoblastoma: longterm tumor control and treatment complications in 208 tumors. Ophthalmology 2001;108:2116-2121.
  • 10. Shields CL, Shields JA. Basic understanding of current classification and management of retinoblastoma. Curr Opin Ophtalmol 2006;17:228-234.
  • 11. Shields CL, Shields JA, Needle M et al. Combined chemoreduction and adjuvant treatment for intraocular retinoblastoma. Ophthalmology 1997;104:2101-2111.
  • 12. Gündüz K, Shields CL, Shields JA et al. The outcome of chemoreduction treatment in patients with Reese- Ellsworth group V retinoblastoma. Arch Ophthalmol 1998;116:1613-1617.