Department of Pediatrics, Beijing Tongren Hospital, Capital Medical University, Yizhuang Economic and Technological Development Zone, No. 2, Xihuan South Road, Beijing, 100176, China.
BMC Cancer. 2024 Mar 4;24(1):287. doi: 10.1186/s12885-024-12058-3.
Management guidelines and corresponding survival data for patients with recurrent retinoblastoma (RB) are lacking. This study aimed to summarize the clinical characteristics of patients with recurrent RB and analyze their survival outcomes.
We retrospectively analyzed 68 patients with recurrent RB who underwent treatment in our institution from January 2016 to December 2020. Patients were grouped according to location of recurrence: intraocular, orbital, and distant metastasis.
The male:female ratio was 1.3:1 and the median age at recurrence was 37.5 months (range, 30.3-62.8). The number of patients in the intraocular recurrence, orbital recurrence, and metastasis groups was 13 (19.1%), 23 (33.8%), and 32 (47.1%), respectively. Thirty patients died, 36 were alive at last follow-up, and two were lost to follow-up. Eye enucleation was performed in 94.1% of patients. Five-year overall survival in patients with intraocular recurrence, orbital recurrence, and metastasis was 84.6%, 69.6%, and 31.3%, respectively (P = 0.001). Most deaths occurred within 2 years of recurrence. Presence of high-risk pathological factors, central nervous system invasion, and absence of combination therapy were independent predictors of worse 5-year overall survival.
The rate of eye preservation in survivors of recurrent RB was very low. Although 5-year overall survival in patients who underwent treatment for intraocular and orbital recurrence was high, it was low in those with metastasis. RB patients may need lifelong follow-up for recurrence and secondary malignancy.
对于复发性视网膜母细胞瘤（RB）患者，缺乏管理指南和相应的生存数据。本研究旨在总结复发性 RB 患者的临床特征，并分析其生存结果。
我们回顾性分析了 2016 年 1 月至 2020 年 12 月在我院接受治疗的 68 例复发性 RB 患者。根据复发部位将患者分组：眼内、眼眶和远处转移。
男女比例为 1.3:1，复发时的中位年龄为 37.5 个月（范围 30.3-62.8）。眼内复发、眼眶复发和转移组的患者分别为 13 例（19.1%）、23 例（33.8%）和 32 例（47.1%）。30 例患者死亡，36 例患者在最后一次随访时仍存活，2 例患者失访。94.1%的患者行眼球摘除术。眼内复发、眼眶复发和转移患者的 5 年总生存率分别为 84.6%、69.6%和 31.3%（P=0.001）。大多数死亡发生在复发后 2 年内。存在高危病理因素、中枢神经系统侵犯和未行联合治疗是 5 年总生存率较差的独立预测因素。
复发性 RB 幸存者保留眼球的比例非常低。尽管接受眼内和眼眶复发治疗的患者 5 年总生存率较高，但转移患者的生存率较低。RB 患者可能需要终生随访以监测复发和继发恶性肿瘤。
