Soft tissue sarcoma as a secondary malignancy after radiotherapy in a child with bilateral retinoblastoma/Bilateral retinoblastom tanisiyla radyoterapi uygulanan bir olguda yumusak doku sarkomu

• Published in: Türk onkoloji dergisi 2011-07, Vol.26 (3), p.121
• Main Authors: Ozdemir, Nihal, Kebudi, Rejin, Hakyemez, Handan Toptan, Ayan, Inci, Agaoglu, Fulya Yaman, Dervisoglu, Sergulen, Darendeliler, Emin
• Format: Article
• Language: Turkish
• ISSN: 1300-7467
• DOI: 10.5505/tjoncol.2011.401

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. Key words: Secondary cancer; retinoblastoma; radiotherapy. Retinoblastom, cocukluk caginda en sik gorulen intraokuler tumordur, ozellikle herediter formlarinda ikincil kanser riski artmistir. Bu olgu sunumunda bilateral herediter retinoblastom tanisiyla cerrahi ve radyoterapi uygulanan hastanin tedaviyi takiben 14 yil sonra radyoterapi uygulanan alanda ortaya cikan yumusak doku sarkomu bildirilmistir. Bir yasinda retinoblastom nedeniyle sag goze enukleasyon, sol goze radyoterapi uygulanan 16 yasinda kiz hasta, 14 yil sonra sol maksiller sinusu dolduran kitle nedeniyle basvurdu. Biyopsi sonucu dusuk dereceli rabdomiyosarkom disi yumusak doku sarkomu olarak rapor edildi. Cerrahi sonrasi kemoterapi uygulanan olguda iki ay icerisinde nuks gelisti ve hasta tekrar ameliyat edildi. Farkli mukerrer kemoterapi rejimleri ve mukerrer ameliyatlar geciren hasta halen hastalikli hayattadir. Herediter retinoblastomlu hastalar ikinci kanserler acisindan risk tasirlar. Radyoterapi alan hastalarda bu risk daha da artmistir. Radyoterapi bolgesinde uzun yillar sonrasinda bile tumor gelisebilecegi unutulmamalidir. Anahtar sozcukler: Ikincil kanser; retinoblastom; radyoterapi.