Leal-Leal Carlos A, Rivera-Luna Roberto, Flores-Rojo Martha, Juárez-Echenique Juan C, Ordaz Juan C, Amador-Zarco Jorge
Department of Oncology, Instituto Nacional de Pediatría, Mexico DF, Mexico.
Clin Transl Oncol. 2006 Jan;8(1):39-44. doi: 10.1007/s12094-006-0093-x.
Retinoblastoma (RB) is the most frequent malignant eye tumor in childhood. In developing countries advanced stages are common. The purpose of this paper is to present our 21-year clinical experience with metastatic extra ocular RB patients treated with 5 different chemotherapy schemas at a single Mexican Pediatric referral center.
A retrospective analysis was carried out reviewing the clinical characteristics of patients with metastatic RB. The information analyzed included the delay in diagnosis after first symptoms, age, sex, ocular staging, and anatomic site of metastases, treatment scheme, initial response and status at the last contact or date of death.
Eighty-one patients were included; age range was from 3 to 80 months. The most common site of metastasis was central nervous system (83.9%). From those patients treated with chemotherapy (n = 74), 89.2% presented a complete initial response (n = 66). Early mortality occurred in 7 cases before any treatment. Fifty-six received treatment and died with progressive disease. All patients without radiotherapy died with tumor activity (n = 15). The use of cisplatin was related with longer disease free intervals; no other variable was related with survival. Four patients were alive and disease free at 33 to 144.3 months of follow up from diagnosis. The prevalent cause of death was tumor progression.
In our experience, metastatic RB has a very high mortality rate in spite of the use of different chemotherapy regimens.
视网膜母细胞瘤（RB）是儿童期最常见的恶性眼肿瘤。在发展中国家，晚期病例很常见。本文旨在介绍我们在墨西哥一家儿科转诊中心，对21年间采用5种不同化疗方案治疗的转移性眼外RB患者的临床经验。
对转移性RB患者的临床特征进行回顾性分析。分析的信息包括首次出现症状后诊断延迟情况、年龄、性别、眼部分期、转移的解剖部位、治疗方案、初始反应以及最后一次随访或死亡日期时的状态。
纳入81例患者；年龄范围为3至80个月。最常见的转移部位是中枢神经系统（83.9%）。在接受化疗的患者中（n = 74），89.2%出现了完全初始反应（n = 66）。7例患者在接受任何治疗前早期死亡。56例接受治疗，死于疾病进展。所有未接受放疗的患者均死于肿瘤活动（n = 15）。顺铂的使用与更长的无病间期相关；没有其他变量与生存相关。4例患者从诊断开始随访33至144.3个月时存活且无疾病。主要死亡原因是肿瘤进展。
根据我们的经验，尽管采用了不同的化疗方案，转移性RB的死亡率仍然很高。
