Malignant transformation of spontaneously regressed retinoblastoma, retinoma/retinocytoma variant.
Malignant transformation of spontaneously regressed retinoblastoma, retinoma/retinocytoma variant.
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DOI：
10.1016/s0161-6420(89)32714-x
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发表时间：
1989-09
期刊：
影响因子：
13.7
通讯作者：
R. Eagle;J. Shields;L. Donoso;Ralph S. Milner
中科院分区：
文献类型：
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作者：
R. Eagle;J. Shields;L. Donoso;Ralph S. Milner
作者报告了一例7岁女孩，4岁时在常规学前筛查中发现单侧视力下降，视网膜病变符合视网膜瘤或视网膜细胞瘤变体的“自发退行性视网膜母细胞瘤”。对病人进行了密切的摄影记录。在保持稳定3年后，肿瘤迅速生长并侵入玻璃体，促使眼球摘除。组织病理学检查显示肿瘤的新隆起部分为未分化的视网膜母细胞瘤，而肿瘤底部具有视网膜瘤/视网膜细胞瘤的典型良性组织病理学特征，包括细胞核淡色，纤维状嗜酸性基质，分散的小花斑，存活肿瘤内的钙化灶，无有丝分裂。免疫组化染色显示，视网膜S抗原、S-100蛋白和胶质原纤维酸性蛋白的免疫反应性局限于肿瘤分化的基底部细胞。这些组织病理学观察支持这样一个概念，即具有患者原始病变临床特征的肿瘤是新生视网膜母细胞瘤的良性变异，而不是自发消退的视网膜母细胞瘤。本病例的临床进展提示视网膜瘤或视网膜细胞瘤在一段时间的临床休眠后很少发生恶性转化，并强调有必要对推定为退行性视网膜母细胞瘤的患者进行密切随访。
The authors report on a case of a 7-year-old girl who was found on routine preschool screening at age 4 years to have unilateral decreased vision and a retinal lesion consistent clinically with the retinoma or retinocytoma variant of “spontaneously regressed retinoblastoma.” The patient was followed closely with photographic documentation. After remaining stable for 3 years, her tumor grew rapidly and seeded the vitreous, prompting enucleation. Histopathologic examination disclosed that the newer elevated part of the tumor was an undifferentiated retinoblastoma, whereas the base of the tumor had the characteristic benign histopathologic features of retinoma/retinocytoma including bland nuclei, a fibrillar eosoinophilic stroma, scattered fleurettes, calcific foci within viable tumor, and no mitoses. Immunohistochemical stains disclosed that immunoreactivity for retinal S antigen, S-100 protein, and glial fibrillary acidic protein was confined to cells in the differentiated basal part of the tumor. These histopathologic observations support the concept that tumors with the clinical characteristics of the patient's original lesion are benign variants of retinobiastomathat have arisen de novo rather than retinoblastomas that have undergone spontaneous regression. The clinical developments in this case suggest that retinomas or retinocytomas rarely undergo malignant transformation after a period of clinical dormancy and underscore the necessity for close follow-up of patients with presumed regressed retinoblastoma.