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¹İstanbul Üniversitesi, Cerrahpaşa Tıp Fakültesi, Pediatrik Hematoloji-Onkoloji Bilim Dalı, İstanbul
²İstanbul Üniversitesi, Onkoloji Enstitüsü, Pediatrik Hematoloji-Onkoloji Bilim Dalı, İstanbul
³İstanbul Üniversitesi, Onkoloji Enstitüsü, Radyasyon Onkolojisi Bilim Dalı, İstanbul
⁴İstanbul Üniversitesi, Cerrahpaşa Tıp Fakültesi, Patoloji Bilim Dalı, İstanbul Retinoblastoma is the most common intraocular tumor in childhood. There is an increased risk of secondary malignancy in these cases, especially in hereditary retinoblastoma. Here, we report a case with bilateral retinoblastoma who was treated with chemotherapy and radiotherapy, and developed soft tissue sarcoma 14 years later within the radiation field. A 16-year-old girl who had enucleation of the right eye and radiotherapy of the left eye when she was 1 year old, presented with a mass in the maxillary sinus. Biopsy was consistent with low-grade non-rhabdomyosarcoma soft tissue sarcoma. The patient was treated with surgery and chemotherapy, but had multiple recurrences. She received various salvage chemotherapy regimens and multiple surgical procedures, and is alive with disease. There is an increased risk of secondary malignancy in retinoblastoma, especially in the hereditary form. This risk is increased in patients receiving radiotherapy. Tumors may appear within the radiation field even years later. Keywords : Secondary cancer; retinoblastoma; radiotherapy