|Year : 1980 | Volume
| Issue : 2 | Page : 97-100
Survival of retinoblastoma cases in North India
SP Dhir1, IS Jain1, GR Dar1, HD Gupta2
1 Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh-160012, India
2 Department of Biostatistics, Postgraduate Institute of Medical Education and Research, Chandigarh-160012, India
S P Dhir
Deptt. of Ophthalmology post graduate Institute of Medical 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 1980;28:97-100
Retinoblastoma is the commonest intraocular tumour seen in India (Gogi and Nath, Dhir et a1). No study is available on the follow up and final outcome of these patients in India. We undertook the present study to find out the factors influencing survival in patients with retinoblastoma.
| Material and methods|| |
47 cases of retinoblastoma, in the period 1972-1976, were taken up for this study. The data on these patients has been analysed. Eleven patients had either insufficient follow up or were lost to follow up. Thus detailed on 36 patients is presented.
| Observations|| |
Age and sex;
The minimum age of presentation was 6 months and the maximum age 7 years. Majority of children (52.7%) presented between the age of 3 and 4 years. Average age of presentation for all cases \ has 4.0 years. There were 28 male patients and 19 female patients in this series. [Table - 1]
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. [Table - 2]
Out of 47 cases, 28 (60%) were unilateral and 19 (40%) were bilateral. Among unilateral cases, right eye was affected in 17 cases and left eye in l 1 cases. In bilateral cases, 15 had more advanced lesion in right eye while in 4 cases, left eye had more advanced lesion. Thus, among the 69 involved eyes the right side was having a lesion in 36 instances and left side in 30 instances. [Table - 3]
In 20 cases (42.5%) enucleation alone was performed while enucleation was combined with radio-therapy or chemotherapy or both in 21 cases (46.8%). In other cases exenteration with or without radiotherapy or chemotherapy was instituted [Table - 4]
| Observations|| |
36 cases out of a total of 47 cases of retinoblastoma could be followed from l-5 years [Table - 1]. A patient surviving for at least a period of one year during follow up, after the initial diagnosis, has been designated as survived.
Age and Sex:
The average age of presentation for the 36 cases followed was 3.5 years. The average age of presentation of children who were alive was 3.1 years and those who have died was 4.0 years. However, this difference was not statistically significant. 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 history of genetic transmission. His sister had died of retinoblastoma whereas his father had a regressed retinoblastoma (Dhir et al,). He died after a period of 6 months follow up. He had bilateral involvement.
Mode of Presentation
Out of 24 cases with proptosis who were followed up, 6 cases (25%) were alive at the end of study. In 7 cases with white pupillary reflex, 4 cases (57%) were alive. All the three cases who presented with squint were alive. The patient with orbital recurrence died in six months time. Patients with orbital involvement had significantly (P<0.01) poor survival than those with intraocular disease alone.
Among the 14 live children 6 cases were unilateral while 8 cases were bilateral. In the unilateral cases, right eye was involved in 5 and left eye in I case. In the bilateral cases, right eye had a more advanced lesion in all cases.
Among the 22 dead children, 14 had unilateral and 8 cases had bilateral involvement. Among the unilateral cases, 1 1 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, [Table - 3]. No significant difference in survival between unilateral and bilateral cases was seen.
Response to Treatment
With enucleation alone in 16 cases, 9 children were alive while seven have died. Among the 7 children who received combined radiotherapy with enucleation, two children were alive while 5 have died. In children where enucleation was followed by both radiotherapy and chemotherapy, one child was alive while 4 have died. In those cases of advanced disease where exenteration, radiotherapy and chemotherapy was instituted, none of the cases survived [Table - 4]. No significant differences were seen in survival 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 6 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.
| Discussion|| |
The average 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 4.0 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 better attention by parents. The factor of hereditary 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 pupillary reflex, squint, intraocular inflammation) had better chances of survival, (73%), than the cases which presented with advanced stages of the disease (proptosis, orbital recurrence), (24%). Mode of presentation as squint is a favourable sign for survival in retinoblastoma.
In the present study bilateral incidence was quite high (40.4%) as compared to that reported in Western literature (36%) (Reese), and Indian Literature, (I1.1%) (Goswamy). It is quite possible that Goswamy based her statistics on histopathological material which will reveal much less bilateral incidence than clinical observations with follow up. 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. In bilateral cases, right eye shows a more advanced lesion. Predominance of right eye involvement was found to have no significant influence on survival.
Simple enucleation was the usual procedure except in advanced disease or those who showed signs of orbital spread. Majority of survived children included those who presented in early stages of the disease and enucleation alone was done. Results after radiotherapy were poor, the reasons being irregular poor follow up and problem of general anaesthesia. Endoxan alone was available as antimitotic drug for chemotherapy. It is difficult to draw any firm conclusion from this study as the patient number is small. Much better comparative studies can be obtained if there is accumulation in one centre in the country of a 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, hereditary, 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|| |
Gogi, R. and Nath K : 1976, East. Arch.Ophthalmol. 3: 138
Dhir, S P.; Jain, I.S. and Das, S.K.: , 1977 Indian J. Ophthalmol. 24 :35, 1977.
Reese, R.A.G. : 1976: Tumours of the eye.Harpert Row Publishers, New York, Third Edition, P 91.
Goswamy, V: , 1975, East. Arch. Ophthalmol. 3: 138
[Table - 1], [Table - 2], [Table - 3], [Table - 4]