Malkani I, Warrier R P, Yu L C, Ode D L
Louisiana State University Medical Centre, Children's Hospital, New Orleans 70118.
Indian J Pediatr. 1993 Mar-Apr;60(2):227-36. doi: 10.1007/BF02822180.
Retinoblastoma is the most common primary ocular malignancy in childhood, usually occurring below the age of five. Recent advances in molecular biology have enabled understanding of retinoblastoma tumorigenesis as well as detecting carriers of the mutant retinoblastoma allele. It is possible now to predict prenatally whether a child carries the retinoblastoma genome. This has enabled early detection of retinoblastoma and improved outcome. Also these children are at risk for mesenchymal tumors in early adult life necessitating life long vigilance. Great strides have been made in the management of retinoblastoma. Introduction of a new pathological staging system and addition of combination chemotherapy for extraocular disease has led to more than 80% long term disease free survivors in this group of patients, who earlier had a very poor outcome. For localized intraocular disease, local treatment seems to be all that is necessary. Newer radiation techniques have helped preserve useful vision and reduced radiation related side effects.
视网膜母细胞瘤是儿童期最常见的原发性眼部恶性肿瘤，通常发生在五岁以下。分子生物学的最新进展使人们能够了解视网膜母细胞瘤的肿瘤发生过程，并检测出携带视网膜母细胞瘤突变等位基因的携带者。现在可以在产前预测儿童是否携带视网膜母细胞瘤基因组。这使得视网膜母细胞瘤能够早期被发现，并改善了治疗结果。此外，这些儿童在成年早期有发生间充质肿瘤的风险，因此需要终身警惕。视网膜母细胞瘤的治疗已经取得了巨大进展。新的病理分期系统的引入以及对眼外疾病采用联合化疗，使得这组患者中超过80%能够长期无病生存，而他们早期的治疗效果非常差。对于局限性眼内疾病，局部治疗似乎就是全部所需。更新的放射技术有助于保留有用视力并减少与放射相关的副作用。
