Donaldson S S, Smith L M
Stanford U Medical School.
Oncology (Williston Park). 1989 Apr;3(4):45-51; discussion 51-2.
Retinoblastoma is an uncommon ocular tumor of childhood which accounts for 5% of childhood blindness. It serves as a prototype for understanding the genetics of childhood cancer. The identification of the retinoblastoma gene has provided an explanation for the differing features of hereditary and nonhereditary retinoblastoma, as well as the potential for secondary malignancies in the hereditary cases. Management decisions are based upon the potential for useful vision, and the extent of disease including whether one or both eyes are involved. As nearly all patients present with disease confined to the globe, local control is excellent and survival exceeds 85%. Goals of management are cure of the disease, preservation of vision, and early detection and treatment of secondary malignancies in the genetically susceptible group.
视网膜母细胞瘤是一种儿童期罕见的眼部肿瘤，占儿童失明病例的5%。它是理解儿童癌症遗传学的一个典范。视网膜母细胞瘤基因的鉴定为遗传性和非遗传性视网膜母细胞瘤的不同特征，以及遗传性病例中发生继发性恶性肿瘤的可能性提供了解释。治疗决策基于保留有用视力的可能性以及疾病的范围，包括是否累及一只或双眼。由于几乎所有患者就诊时疾病都局限于眼球内，局部控制效果良好，生存率超过85%。治疗目标是治愈疾病、保留视力，以及在遗传易感性人群中早期发现和治疗继发性恶性肿瘤。
