眼外视网膜母细胞瘤：13年的经验

Extraocular retinoblastoma: a 13-year experience.

作者信息

Antoneli Célia Beatriz Gianotti, Steinhorst Flávio, de Cássia Braga Ribeiro Karina, Novaes Paulo Eduardo R S, Chojniak Martha M M, Arias Victor, de Camargo Beatriz

机构信息

Department of Pediatric Oncology, Hospital do Câncer, A. C. Camargo, Centro de Tratamento e Pesquisa, São Paulo, Brazil.

出版信息

Cancer. 2003 Sep 15;98(6):1292-8. doi: 10.1002/cncr.11647.

DOI:10.1002/cncr.11647

PMID:12973854

Abstract

BACKGROUND

The current study was performed to evaluate two regimens of treatment and to describe clinical and epidemiologic characteristics in patients with extraocular retinoblastoma.

METHODS

Eighty-three patients with extraocular retinoblastoma according to Childrens Cancer Group (CCG) classification were admitted to the Pediatric Department of the A. C. Camargo between 1987-2000. The age, gender, race, lag time, first clinical presentation, staging, laterality, and treatment regimen were analyzed. Treatment was comprised of cisplatin, teniposide, vincristine, doxorubicin, and cyclophosphamide during the first treatment period (1987-1991) or cisplatin and teniposide with alternating courses of ifosfamide and etoposide during the second treatment period (1992-2000).

RESULTS

The mean age of the patients was 32.9 months (range, 2-145 months). The mean lag time was 10.5 months. Forty-three patients were treated in the first period and 40 patients were treated in the second period. Locally advanced tumors (Class I-III) were present in 83.1% of the patients. There was a positive correlation between lag time and age for unilateral tumors (correlation coefficient [r] = 0.35; P = 0.006), whereas the correlation was negative for bilateral tumors (r = -0.12; P = 0.63). The 5-year overall survival was 55.1% in the first treatment period and 59.4% in the second treatment period (P = 0.69). No significant differences with regard to survival rates were noted for unilateral tumors between the two treatment periods (44.6 noted for unilateral tumors vs. 59.1 noted for unilateral tumors).

CONCLUSIONS

In the current study, the addition of ifosfamide and etoposide to a treatment regimen comprised of cisplatin, teniposide, vincristine, doxorubicin, and cyclophosphamide did not appear to improve the survival of patients with extraocular retinoblastoma. Patients with dissemination to the central nervous system or metastatic disease remain incurable and die of progressive disease, despite the aggressive treatment. A multicenter trial should be considered to evaluate the best strategy for these situations.

摘要

背景

本研究旨在评估两种治疗方案，并描述眼外视网膜母细胞瘤患者的临床和流行病学特征。

方法

1987年至2000年间，83例根据儿童癌症组（CCG）分类的眼外视网膜母细胞瘤患者被收治于A.C.卡马戈儿科。分析了患者的年龄、性别、种族、延迟时间、首次临床表现、分期、病变侧别和治疗方案。在第一个治疗期（1987 - 1991年），治疗包括顺铂、替尼泊苷、长春新碱、阿霉素和环磷酰胺；在第二个治疗期（1992 - 2000年），治疗包括顺铂和替尼泊苷，联合异环磷酰胺和依托泊苷交替疗程。

结果

患者的平均年龄为32.9个月（范围为2 - 145个月）。平均延迟时间为10.5个月。43例患者在第一期接受治疗，40例患者在第二期接受治疗。83.1%的患者存在局部晚期肿瘤（I - III级）。单侧肿瘤的延迟时间与年龄呈正相关（相关系数[r]=0.35；P = 0.006），而双侧肿瘤的相关性为负（r = -0.12；P = 0.63）。第一个治疗期的5年总生存率为55.1%，第二个治疗期为59.4%（P = 0.69）。两个治疗期的单侧肿瘤生存率无显著差异（单侧肿瘤分别为44.6%和59.1%）。