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Abstract
Purpose
To determine the incidence (1991∼1993) and survival rates (1991∼2003) of retinoblastoma in Korea.
Methods
Data of all Korean patients diagnosed with retinoblastoma from 1991–1993 were retrospectively collected by the hospi-tal-based national registry based on notifications from physicians who are able to diagnose retinoblastoma. The incidence rate (1991∼1993) was calculated per 100,000 childbirths (dividing the number of retinoblastoma patients by the total number of childbirths). The survival rate (1991∼2003) was calculated using the Kaplan– Meier method, and the differences were evaluated with the logrank and Wilcoxon tests and Cox's proportional hazards model. In addition, the clinical findings and prognostic factors associated with survival rate were analyzed.
Results
There were a total of 108 distinct patients diagnosed with retinoblastoma in the registry. The incidence of retinoblastoma in Koreawas 4.99 per 100,000 childbirths, and the survival rate for 13 years (1991–2003) was 77.8%. The survival rate was significantly lower in the population of bilateral retinoblastomapatients, as compared to unilateral retinoblastoma patients. Optic nerve invasion was found to be an independent variable for the reduction of survival rate, although its effects were not significant.
Conclusions
In the present study, the incidence and survival rates of retinoblastoma in Koreawere presented for the first time. The incidence rate was similar to rates reported from other countries. On the other hand, the survival rate was lower than those in European and the United States. In particularly, the survival rate was significantly lower in patients with bilateral retinoblastoma.
References
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Figure 2.
Data ordered by the length of observed survival time, with (D) representing dead and (A) alive at the end of the follow-up period.
Figure 3.
Survival curve produced by the Kaplan-Meier method for the 108 retinoblastoma patients according to age at diagnosis (≤1 y vs. 2 y ≤).
Figure 4.
Survival curve produced by the Kaplan-Meier method for the 108 retinoblastoma patients according to sex.
Figure 5.
Survival curve produced by the Kaplan-Meier method for the 108 retinoblastoma patients according to bilateral status.
Figure 6.
Survival curve produced by the Kaplan-Meier method for the 108 retinoblastoma patients according to operation status.
Figure 7.
Survival curve produced by the Kaplan-Meier method for the 108 retinoblastoma patients according to optic nerve infiltration.
Table 1.
Prognostic factors for retinoblastoma in Korea (1991∼1993)
|
Crude |
Adjusted* |
Adjusted† |
|||
|---|---|---|---|---|---|
| Dead n (%) | Alive n (%) | RR (95%CI) | RR (95%CI) | RR (95%CI) | |
| Age at diagnosis | |||||
| ≤1y | 14 (20.3) | 55 (79.7) | 1 | 1 | 1 |
| ≤2y | 10 (25.6) | 29 (74.4) | 1.32 (0.59–2.98) | 1.88 (0.79–4.45) | 1.92 (0.74–4.97) |
| Sex | |||||
| Male | 7 (14.9) | 40 (85.1) | 1 | 1 | 1 |
| Female | 17 (27.9) | 44 (72.1) | 2.09 (0.87–5.05) | 2.21 (0.91–5.36) | 2.28 (0.85–6.10) |
| Bilaterality‡ | |||||
| unilateral | 14 (16.9) | 69 (83.1) | 1 | 1 | 1 |
| bilateral | 10 (40.0) | 15 (80.2) | 2.62 (1.16–5.89) | 3.15 (1.33–7.44) | 3.20 (1.19–8.66) |
| Operation | |||||
| - | 5 (41.7) | 7 (58.3) | 1 | – | – |
| + | 19 (19.8) | 77 (80.2) | 0.38 (0.14–1.02) | – | – |
| Optic nerve invasion | |||||
| - | 7 (13.2) | 46 (86.8) | 1 | – | 1 |
| + | 12 (27.9) | 31 (72.1) | 2.39 (0.94–6.07) | – | 2.39 (0.94–6.10) |
Table 2.
Reese-Ellsworth classification of 108 retinoblastoma patients (eyes)
| R-E class |
1 |
2 |
3 |
4 |
5 |
unspecified | |||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| a | b | a | b | a | b | a | b | a | b | ||
| Bilateral | |||||||||||
| Rt (25) | 1 | 1 | 2 | – | – | 2 | – | 1 | 8 | 7 | 3 |
| Lt (25) | 2 | 2 | 3 | 1 | 3 | 1 | – | 1 | 4 | 3 | 5 |
| Unilateral (83) | 1 | – | 13 | 2 | 1 | 3 | – | 3 | 24 | 23 | 13 |
| Total | 4 | 3 | 18 | 3 | 4 | 6 | – | 5 | 36 | 33 | 21 |
Table 3.
Types of treatment for 108 retinoblastoma patients (no)
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