Wilson Matthew W, Haik Barrett G, Billups Catherine A, Rodriguez-Galindo Carlos
Department of Ophthalmology/Hamilton Eye Institute, University of Tennessee Health Science Center, Memphis, Tennessee 38163, USA.
Ophthalmology. 2007 Nov;114(11):2077-82. doi: 10.1016/j.ophtha.2007.03.015. Epub 2007 Jul 12.
To report the incidence of new tumor formation in hereditary retinoblastoma patients treated with primary systemic chemotherapy.
Noncomparative retrospective case series.
Fifty-eight consecutive patients with hereditary retinoblastoma treated with primary systemic chemotherapy.
The charts of 58 consecutive patients with hereditary retinoblastoma treated between January 1996 and August 2005 were reviewed. Data extracted included gender, age at diagnosis, family history of retinoblastoma, laterality of disease, tumors per eye, Reese-Ellsworth grouping of affected eyes, starting and ending dates for chemotherapy, number of cycles of chemotherapy, chemotherapy regimen, need for external beam radiotherapy and/or enucleation, and development and location (macula, midzone, and periphery) of new tumors after the start of systemic chemotherapy.
New tumor formation after treatment with primary systemic chemotherapy.
Of the 58 patients, 48 had bilateral involvement at diagnosis. Median age at diagnosis was 6.6 months. Thirteen patients had a positive family history. Of the eyes with tumor (n = 106) at diagnosis, 52 (49%) were in Reese-Ellsworth groups I to III, whereas 54 (51%) were in group IV or V. Seven patients (12%) with a median age of 1.6 months at diagnosis formed 36 new tumors in 11 eyes after the start of chemotherapy. Median time from initiation of chemotherapy to detection of the first new tumor was 3 months (range, 1-15). Cumulative incidence of new tumor formation at 2 years was 10+/-3%. An age of <6 months at diagnosis, family history of retinoblastoma, and Reese-Ellsworth grouping of I to III were found to correlate significantly with an increased incidence of new tumor formation (P<0.001, P<0.001, and P = 0.021, respectively). Median follow-up for all patients was 5 years (range, 1-10.1).
New tumors continue to form in patients with hereditary retinoblastoma despite treatment with primary systemic chemotherapy. Younger patients and those with a positive family history are more likely to have new tumors formed. However, chemotherapy may impact small previously undetected lesions by slowing their growth and facilitating later focal consolidation.
报告接受原发性全身化疗的遗传性视网膜母细胞瘤患者中新发肿瘤形成的发生率。
非对照性回顾性病例系列。
58例连续接受原发性全身化疗的遗传性视网膜母细胞瘤患者。
回顾了1996年1月至2005年8月期间连续治疗的58例遗传性视网膜母细胞瘤患者的病历。提取的数据包括性别、诊断时年龄、视网膜母细胞瘤家族史、病变侧别、每眼肿瘤数量、患眼的里斯-埃尔斯沃思分级、化疗开始和结束日期、化疗周期数、化疗方案、是否需要外照射放疗和/或眼球摘除，以及全身化疗开始后新肿瘤的发生情况和位置（黄斑、中区和周边）。
原发性全身化疗治疗后新发肿瘤形成情况。
58例患者中，48例诊断时为双侧受累。诊断时的中位年龄为6.6个月。13例患者有阳性家族史。诊断时患肿瘤的眼（n = 106）中，52只（49%）属于里斯-埃尔斯沃思I至III组，而54只（51%）属于IV或V组。7例患者（12%）诊断时的中位年龄为1.6个月，化疗开始后在11只眼中形成了36个新肿瘤。从化疗开始到检测到第一个新肿瘤的中位时间为3个月（范围1 - 15个月）。2年时新发肿瘤形成的累积发生率为10±3%。发现诊断时年龄<6个月、视网膜母细胞瘤家族史以及里斯-埃尔斯沃思I至III组与新发肿瘤形成发生率增加显著相关（分别为P<0.001、P<0.001和P = 0.021）。所有患者的中位随访时间为5年（范围1 - 10.1年）。
尽管接受了原发性全身化疗，遗传性视网膜母细胞瘤患者仍会继续形成新肿瘤。年龄较小的患者和有阳性家族史的患者更有可能形成新肿瘤。然而，化疗可能通过减缓小的先前未检测到的病变生长并促进后期局部巩固来影响它们。
