Clinical analysis of 2790 children with retinoblastoma: a single-center experience in China

Background In this study,we aimed to analyze the clinical characteristics and prognosis of children with retinoblastoma(RB)in a single center in China with a large sample collection spanning 17 years.Methods The clinical data of 2790 children with RB treated in Beijing Tongren Hospital from 2005 to 2021 were collected,and a retrospective analysis was conducted.Results The median age of the participants was 28.3 months.There were 3624 affected eyes,12.4%of which were in groups A–C,67.1%in groups D–E and 16.2%were not specified.The primary symptom observed in most cases was a white pupil,accounting for 66.5%,followed by strabismus(12.8%).The median follow-up time was 59.7 months.The enucleation rate was 71.3%(703/986)in a single left eye and 72.5%(702/968)in a single right eye.The overall survival(OS)rate was 95.8%(2444/2552)because 237 patients dropped out,and 109 died.Kaplan‒Meier survival analysis showed that the median survival time(MST)was 125.92 months[95%confidence interval(CI)=124.83–127.01].Cox multivariate survival analysis showed that trilateral RB(P=0.017),metastasis site(P=0.001),and combined distant tissue metastasis(P=0.001)were independent prognostic factors for RB.The OS of 44 cases of familial RB was 93.2%(41/44),with an MST of 80.62 months(95%CI=67.70–93.54).Conclusions The timing of eye protection treatment and enucleation should be comprehensively judged to avoid worsening prognosis due to operation time delay.More importantly,the promotion and popularization of diagnosis and treatment technologies are necessary to further improve RB prognosis.

Telomerase expression in sebaceous carcinoma of the eyelid

Background In humans telomerase is expressed in most ca ncers and immortal cell lines, and astivation of telomerase may play important r oles in tumorigenesis and immortalization. This study was to investigate the rol es of telomerase activity (TA) and human telomerase RNA (hTR) in sebaceous carcinoma of the eyelid.Methods The telomerase repeated amplification protocal (TRAP) was used to demonstrate telomerase activity in 12 cases of sebaceous carcinoma of the eyelid. In situ hybridiza tion (ISH) was used to demonstrate the expression of hTR in 55 cases of paraffin-embedded sebaceous carcinoma of the eyelid, and the results were compared with the proliferative index determined by Mib-1 immuno-labeling, histological patterns and r ecurrence of the tumor.Results Different telomerase activity was shown in the 12 cases of sebaceous carcinoma of the eyelid. The positive expression of hTR was 85.5% (47/55) in tumor cells, but not in the adjacent tissues. The positive expression of hTR was correlated with the proliferative activity (as assessed by Mib-1 immunolabelling, r=0.942, P<0.001) and the dif ferentiation of sebaceous carcinoma of the eyelid (χ 2=17.621, P<0.001), but not si gnificantly related to tumor recurrence. The level of hTR expression increased with the de crease of differentiation of sebaceous carcinoma of the eyelid.Conclusions The results suggest that the up-regulation of telo merase expression plays some roles in tarsal gland carcinogenesis, and the express ion of hTR is a useful marker for malignant degree of sebaceous carcinoma of the eyelid.