|Year : 1981 | Volume
| Issue : 4 | Page : 447-450
Survival of retinoblastoma cases in North India
SP Dhir, IS Jain, GR Dar, HD Gupta
Department of Ophthalmology and Biostatistics, Postgraduate Institute of Medical Education and Research, Chandigarh, India
I S Jain
Department of Ophthalmology and Biostatistics, Postgraduate Institute of Medical Education and Research, Chandigarh
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Dhir S P, Jain I S, Dar G R, Gupta H D. Survival of retinoblastoma cases in North India. Indian J Ophthalmol 1981;29:447-50
|How to cite this URL:|
Dhir S P, Jain I S, Dar G R, Gupta H D. Survival of retinoblastoma cases in North India. Indian J Ophthalmol [serial online] 1981 [cited 2020 May 28];29:447-50. Available from: http://www.ijo.in/text.asp?1981/29/4/447/30951
Retinoblastoma is the commonest intraocular tumour seen in India,. No study is available on the follow up, and final outcome in these patients in India. We undertook the present study to final out the factors influencing survival in patients with retinoblastoma.
| Materials and methods|| |
47 cases of retinoblastoma were admitted in Eye Department of Nehru Hospital, attached to the Postgraduate Institute of Medical Education & Research, Chandigarh, during the period 1972-1976. These cases have been followed for 1-5 years. The data on these patients has been analysed. Eleven patients had either insufficient follow up or were lost to follow up. Thus data on 36 patients is presented.
| Observations|| |
Age and Sex : Age and sex distrbiution of patients admitted and followed is shown in [Table - 1]. The minimum age of presentation was 6 months and the maximum age was 7 years. Majority of children (52.7%) presented between the age of 3 and 4 years. Average age of presentation for all cases was 3.5 years, for those who were alive was 3.1 years and for those who died was 4 years. However, this difference was not statistically significant. In all 28 cases were males and 19 cases females.
The sex ratio in cases followed was 20:16, for those who were alive, sex ratio was 9:5, while for those who died, sex ratio was 11:11. Male children survived more often than female children. However, it was not statistically significant. The duration of survival also did not differ significantly in the two sexes.
Only one case had a regressed retinoblastoma in the father and his sister had died of retinoblastoma. He died after a period of 6 months follow up. He had bilateral involvement.
Mode of presentation
Proptosis was the most common mode of presentation. Out of 47 cases, 32 cases (68%) presented with proptosis. Eight cases (17%) presented with white pupillary reflex, 3 cases (6.1%) with squint, 3 cases (6.1%) with orbital recurrence and one case (2.8%) with inflammation of the eye as shown in [Table - 2].
Mode of presentation and survival
Out of 24 cases with proptosis followed 6 cases (25%) survived. In 7 cases with white pupillary reflex, 4 cases (57%), survived. All the three cases who presented with squint, survived. Patient with orbital recurrence who was followed died in six months time. Patients with orbital involvement had significantly (P <0.01) poor survival than those with intra, ocular disease alone.
Out of 47 cases, 28 (60%) were unilateral and 19 (40%) cases were bilateral. Among unilateral cases, right eye was affected in 17 cases and left eye in 11 cases. In bilateral cases, 15 had more advanced lesion in right eye while in 4 cases, left eye had more advanced lesion.
Ocular involvement and survival:
Among the alive children, 6 cases were unilateral while 8 cases were bilateral. In the unilateral cases, right eye was involved in 5 and left eye in 1 case. In the bilateral cases, right eye had advanced lesion in all cases.
Among the dead children, 14 cases were unilateral, while 8 cases were bilateral. In the unilateral cases, 11 cases had right eye involvement and 3 cases had left eye involvement. In bilateral cases, right eye had more advanced lesion in 6 cases and left eye in 2 cases, as shown in [Table - 3]. No significant difference in survival between unilateral and bilateral cases was seen.
In 20 cases (42.5%) enulcleation alone was performed while enucleation was combined with radio-therapy or chemotherapy or both in 21 cases (46.8%). In other cases, exentration, with or without radiotherapy or chemotherapy was instituted as shown in [Table - 4].
Relation of treatment to survival
With enucleation alone in 16 cases, 9 children survived while seven died. Among the seven children who had combined radiotherapy with enucleation, 2 children survived while 5 cases died. Fifteen children where enucleation was followed by both radio-therapy and chemotherapy, one case survived while 4 cases died. In those cases where exentration, radiotherapy and chemotherapy was instituted, none of the cases survived as seen in [Table - 4]. No significant difference was seen in survivlal between various modes of therapy.
Status of survivors
Of the 14 cases who were alive at the end of study, 6 were unilateral cases and 8 were bilateral cases. There were four cases free of disease out of 6 unilateral survivors. Two had orbital recurrences. Of the 8 bilateral cases who were alive, two were free of disease, 3 had controlled disease and three had advanced disease. The two bilateral cases who were free from disease had enucleation in one eye and a lesion 1 /4 to 1 /2 size of disc was detected in the other eye which completely disappeared following radio-therapy.
| Comments|| |
The average age of presentation was older than reported previously in the literature. Reese reported average age of presentation as 13 months. In the present study it was 4.3 years.
The children who were alive presented at earlier age (3.1 years) than those who died (4.0 years). The presentation at older age is associated with more advanced disease. 'This partly accounts for poor rate of survival in India than reported in western countries. Male children had more chances of survival than female children perhaps due to bettor attention by parents. The factor of heredity in survival could not be studied as there was only one case.
The children who presented with disease limited to the interior of the eyeball (white pupilary reflex, squint, intraocular inflammation) had good chances of survival (73%), that the cases presented with advanced stages of the disease (proptosis, orbital recurrence) (24%). Mode of presentation as squint is a favourable sign for survival in retinoblastoma. Patients with diseasa limited to the inside the eye had significantly better chance of survival than those with spread to orbital tissues.
In the present study bilateral incidence was quite high (40.4%) as compared to the report in westorn literature (37%). However, it was seen that bilateral involvement did not influence the survival of the patient in this study. In bilateral cases the lesion in the eye with more advanced disease governed the survival.
Right eye involvement in retinoblastoma is more commonly seen than left eye both in unilateral and bilateral cases. Even in bilateral cases, right eye bears more frequently the advanced lesion. Predominance of right eye involvement was found to have no significant influence on survival.
Simple enucleation was usual procedure except in advanced disease of those who showed signs of orbital spread. Majority of survived children included those who presented in early stage of disease and enucleation alone was done. Results after radiotherapy were poor, the reasons being irregular follow up and problems of general anaesthesia. Endoxan alone was available as antimitotic drug chemotherapy. It is difficult to draw any firm conclusions from study as patient number is small. Much better comparative studies can be obtained if there is accumulation in one centre in the country of large number of patients with retinoblastoma.
| Summary|| |
Of the 47 cases of histopathologically proven retinoblastoma admitted in PGI, 36 cases have been followed for 1-5 years. The data has been analysed as to age, sex, heredity mode of presentation ocular involvement and treatment in relation to survival of these patients. It was found that mode of presentation significantly affected survival of children with retinoblastoma.
| References|| |
Dhir, S. P. Jain, I S. and Jain, G.C. 1977, Ind. Paediat , 14: 475. 1977.
Dhir, S.P. Jain, I.S. and Das, S.K, 1977, Ind. J. Ophthalmol 24:35.
Gogi, R. and Nath K, 1975, East. Arch. Ophthalmol 3:138.
Reese, A.G, 1976, Tumours of the eye. Harper Row Publishers, New York, Third Edition P. 91.
[Table - 1], [Table - 2], [Table - 3], [Table - 4]