Grabowski E F, Abramson D H
Division of Pediatric Hematology/Oncology, New York Hospital, New York.
Hematol Oncol Clin North Am. 1987 Dec;1(4):721-35.
Retinoblastoma, the most common primary ocular malignancy of childhood, is a tumor in which the pediatrician and pediatric oncologist can now play a much more significant role in therapy. Developments in molecular biology have now made carrier testing and prenatal diagnosis feasible. In the near future, these developments should greatly augment the pediatrician's and pediatric oncologist's ability to offer accurate and appropriate genetic counseling for affected families. A practical staging system for extraocular retinoblastoma together with stage-related effective chemotherapy and radiation therapy was presented in this chapter. These modalities now make possible long-term survival for the majority of the 1 out of 8 children with retinoblastoma who would otherwise die from metastatic disease. Finally, 40 per cent of all children with retinoblastoma (those with the germinal mutation) are at lifelong risk for second, nonocular malignancies. The recognition that more than half of these children will actually develop second tumors by the fourth decade of life makes vigilant follow-up care for these patients a necessity.
视网膜母细胞瘤是儿童期最常见的原发性眼部恶性肿瘤，在这种肿瘤的治疗中，儿科医生和儿科肿瘤学家如今能够发挥更为重要的作用。分子生物学的发展现已使携带者检测和产前诊断成为可能。在不久的将来，这些进展应能极大地增强儿科医生和儿科肿瘤学家为受影响家庭提供准确且恰当的遗传咨询的能力。本章介绍了一种针对眼外视网膜母细胞瘤的实用分期系统以及与分期相关的有效化疗和放射治疗方法。这些治疗方式如今使得每8名视网膜母细胞瘤患儿中有1名（这些患儿若不接受治疗会死于转移性疾病）中的大多数能够长期存活。最后，所有视网膜母细胞瘤患儿中有4０%（即那些有胚系突变的患儿）终生有患第二种非眼部恶性肿瘤的风险。认识到超过一半的这些患儿在40岁时实际上会患上第二种肿瘤，使得对这些患者进行警惕的随访护理成为必要。
