| 발표일자: |
2015년 11월 6일(금) ~ 11월 8일(일) |
| 발표번호: |
P(e-poster)-251 |
| 발표장소: |
킨텍스 제2전시장 7B홀 |
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| 가족력이 없는 일측성 망막모세포종 환자에서 발생한 눈꺼풀의 이차성 원발 횡문근육종 |
| 서울대학교 의과대학 안과학교실(1), 서울대학교병원 안과 (2), 서울시립보라매병원 안과(3), 분당서울대학교병원 안과(4) |
| 문자윤(1,2), 정호경(1,3), 류영주(1,2), 김남주(1,4), 곽상인(1,2) |
| 본문 : It has been reported that the hereditary form of retinoblastoma or retinoblastoma with RB gene mutations has increased risk of second malignancy development, especially when radiation therapy is involved in the treatment. There hasn’t been yet a case report of second malignancy in nonhereditary unilateral retinoblastoma worldwide. We report a 11 year old patient with nonhereditary unilateral retinoblastoma without any gene abnormalities who received enucleation and chemotherapy but developed pure eyelid rhabdomyosarcoma. Seven years after chemotherapy completion, the patient developed a slow growing painless polypoid mass confined to the eyelid margin and immunohistochemistry findings were positive for desmin and myogenin, which are specific markers for rhabdomyosarcoma. The patient was treated with chemotherapy after wide surgical excision. This case report is the first to report a patient with second primary rhabdomyosarcoma after treatment of retinoblastoma with enucleation and chemotherapy, without radiation therapy. We recommend consideration of rhabdomyosarcoma confined to the eyelid in diagnosing eyelid mass in retinoblastoma patients. Also further studies in the relation between chemotherapy and rhabdomyosarcoma development, and retinoblastoma related second primary malignancies should be performed. In addition, investigation of other possible gene mutations, other than the RB gene mutation, that may affect the occurrence of second primary malignancies should be conducted. |
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