Roelofs Kelsey, Kherani Femida, Russell Laurie, Heathcote J Godfrey, Weis Ezekiel
Department of Ophthalmology and Visual Sciences.
Department of Surgery, University of Calgary, Calgary, AB.
Ocul Oncol Pathol. 2017 Nov;3(4):296-300. doi: 10.1159/000468523. Epub 2017 Apr 14.
In 1974, an 8-month-old male was diagnosed with bilateral retinoblastoma. His left eye was enucleated, while the right eye was salvaged with a combination of external beam radiotherapy (4,000 cGy total, divided in 20 fractions) and retinal laser treatment. Thirty-nine years later, he developed intraocular recurrence of retinoblastoma with extrascleral spread. Histopathological examination also identified a second distinct malignancy, retinal pigment epithelium adenocarcinoma, arising in continuity with the retinoblastoma. Further investigation revealed foci of metastatic retinoblastoma in his parotid gland. He was subsequently treated with a combination of orbital exenteration, extensive neck dissection, and resection of metastatic foci, followed by a high-dose ablative chemotherapeutic regimen consisting of cisplatin, vincristine, and cyclophosphamide. Although very rare, late recurrence of retinoblastoma with systemic metastasis is possible, and continued clinical observation and appropriate long-term follow-up should be considered. Additionally, it is important to consider a second primary intraocular tumor in the differential diagnosis, especially in a patient with heritable retinoblastoma who has undergone radiation therapy.
1974年，一名8个月大的男性被诊断为双侧视网膜母细胞瘤。他的左眼被摘除，而右眼通过外照射放疗（总量4000 cGy，分20次进行）和视网膜激光治疗得以保留。39年后，他发生了视网膜母细胞瘤眼内复发并伴有巩膜外扩散。组织病理学检查还发现了第二种不同的恶性肿瘤，即视网膜色素上皮腺癌，与视网膜母细胞瘤连续发生。进一步检查发现他的腮腺有转移性视网膜母细胞瘤病灶。随后，他接受了眶内容摘除术、广泛的颈部清扫术和转移灶切除术，接着进行了由顺铂、长春新碱和环磷酰胺组成的高剂量消融化疗方案。尽管非常罕见，但视网膜母细胞瘤晚期复发并伴有全身转移是有可能的，应考虑持续的临床观察和适当的长期随访。此外，在鉴别诊断中考虑第二种原发性眼内肿瘤很重要，特别是在接受过放射治疗的遗传性视网膜母细胞瘤患者中。
