Clinical characteristics and prognosis analysis of 217 infant patients with retinoblastoma / 中华实用儿科临床杂志

ABSTRACT

Objective:To investigate the clinical characteristics and prognosis of infants with retinoblastoma (RB).Methods:The clinical data of 217 (335 affected eyes) infantile RB patients admitted to the Department of Pediatrics, Beijing Tongren Hospital, Capital Medical University from July 1, 2009 to June 30, 2019 were collected for analyzing the clinical efficacy and prognosis after comprehensive treatment.Results:(1) Clinical characteristics: 217 infantile RB patients included 129 males and 88 females, with the ratio of male to female being 1.47∶1.00; the median age was 6.06 months; there was monocular disease in 99 cases, and binocular disease in 118 cases, with the incidence ratio of binocular to monocular disease being 1.19∶1.00; all the 5 cases with family history of RB had binocular disease; the first symptoms included white pupils and yellow-white reflections in pupils (183 cases, 84.3%), followed by strabismus (18 cases, 8.3%). There were 335 affected eyes, of which 304 counts were in the intraocular stage (90.7%), most commonly in stage D (146 counts, 43.6%); 26 counts (7.7%) in the extraocular stage, mainly invading the optic nerve and/or optic nerve stump; 5 counts (1.5%) in the metastasis stage.(2) Survival analysis: the medical follow-up continued to March 31, 2020, with a median follow-up time of 67 months.There were 2 cases with a loss of follow-up, 21 relapsed cases, and 19 death cases, with the overall survival rate being 91.2%.According to Kaplan-Meier survival analysis, the expected 5-year survival rate was 91.1%; the survival rate was 96.2% in the intraocular stage and 73.1% in the extraocular stage.All 5 cases died during the distant metastasis stage, and the difference was statistically significant ( χ2=7.492, P<0.001); there was also a statistical difference in the survival rate between the monocular disease (95.9%) and binocular disease (87.3%) ( χ2=4.335, P=0.023). (3) Eyeball removal and eye protection: the eye protection rate of 217 children was 68.9%, 100.0% in stage A, B and C, 80.1% in stage D and 35.1% in stage E, which showed significant differences ( χ2=6.573, P=0.004). There were 35 children who underwent eye extraction before chemotherapy and 67 cases after chemotherapy, among which the difference was statistically significant ( χ2=6.076, P=0.012) in eye removal rates before and after chemotherapy in stage D and E(6 and 11 cases before chemotherapy, 22 and 36 cases after chemotherapy). (4) Adverse reactions: according to the World Health Organization′s classification of adverse reactions to chemotherapy, there were 26 cases in grade 0 (12.0%), 98 cases in grade Ⅰ (45.1%), 59 cases in grade Ⅱ (27.2%), 23 cases in grade Ⅲ (10.6%), and 11 cases in grade Ⅳ (5.1%), mainly manifested as bone marrow suppression after chemotherapy (132 cases). No second tumors appeared, and only 4 cases had transient hearing abnormalities, which returned to the normal state in the subsequent review. Conclusions:Infantile RB has its own characteristics.Such factors as different eye types and different clinical stages can affect the prognosis of children.The survival rate of such children can be improved significantly and the rate of eyeball removal can be reduced after treatment.