|Year : 1964 | Volume
| Issue : 3 | Page : 128-131
Some observations on retinoblastoma
Santi Pada Das
Eye Infirmary, Medical College Hospitals, Calcutta, India
|Date of Web Publication||13-Feb-2008|
Santi Pada Das
Eye Infirmary, Medical College Hospitals, Calcutta
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Das SP. Some observations on retinoblastoma. Indian J Ophthalmol 1964;12:128-31
In the Eye Infirmary, Medical College Hospitals, Calcutta, over a period of eleven years (1950-1960), 590 cases of tumours of the eye were examined histopathologically. Of these, 140 were those of retinoblastoma, forming 23.75 per cent of all the tumours of the eye.
| Clinical Data|| |
Age- The average age of the present series of cases was 3.5 years, the youngest being three weeks old and the oldest 20 years of age. Only seven patients of this series were over 10 years old.
Sex-77 cases were males and the rest 63 were females.
Eyes Involved- The disease was bilateral in 38 cases i.e. in 27 per cent. In 62 cases the right eye was affected and in 59 the left eye was involved.
Heredity-All the cases of the present series were sporadic, except one where the mother was a retinoblastoma survivor. The mother's eye was enucleated for retino-blastoma when she was two years old. She had two female issues. The eye of her first daughter was enucleated for retinoblastoma when the child was one and a half years old. She is now 5 years and 4 months and is doing well at present. Her second daughter when she was six months old had enucleation of the right eye and is doing well for the last three years.
Three instances of familial incidence where the parents were free from the disease were noted in this series. In the first family, three children out of seven were affected. In the second family, two children out of five suffered from the disease and in the third family none of the five children suffering from retinoblastoma survived.
Relation to Injury- Some association of the disease with injury was found in seven of the present cases. For example, one patient aged 4 years was brought to this institution with intraocular growth bulging through the perforated cornea of the right eye. The mother said that her child fell on the ground and then she noticed the big swelling. Before injury the eye had appeared normal. There was no history of a yellow reflex from the pupil. On histopathological examination, section showed the picture of retinoblastoma with extraocular extension. The cornea was perforated and the growth was protruding out through the perforated cornea.
| Clinico-Pathological Data|| |
Rosette Formation was noted histologically in 6 per cent of cases and in the rest, rosette formation was very little or none.
Invasion of the Optic Nerve was discovered in 64 per cent of cases. The size of the tumour had no relation to the degree of optic nerve extension. Occasionally the tumour was small but had already invaded the optic nerve. Again, it was sometimes found that the tumour was quite big with extraocular extension but the optic nerve had not been invaded by the tumour. This happened in four of the present cases. A patient aged two years came to the institution with a bulging left eye. During operation it was found that there was a big retrobulbar mass attached to the posterior aspect of the eyeball. The histopathological report was retinoblastoma with extraocular extension but the optic nerve was not infiltrated by the tumour cells. In seven cases the tumour had invaded the lumen of the central vessels. The choroid was found involved by the tumour in 34 per cent cases.
Spontaneous Retrogression was noted in three cases where the eyes resulted in phthisis bulbi. Histologically calcium deposition was seen in the areas of necrosis and fibrous tissue was abundant. Individual cell necrosis, scattered areas of calcified retinoblastoma cell and even occasionally bone formation were also seen. One of the patients aged one and a half years came to the institution with complaint of swelling, redness and steamy cornea of the left eye. The parents noticed the same features in the right eye of the child after birth. All the features of the right eye of the child subsided gradually without treatment and the right eye gradually became phthisic. The right eye was enucleated this time and the histopathological appearance showed the picture of spontaneous retrogression of retinoblastoma with calcified deposits and phthisis bulbi of the right eye.
The treatment adopted was enucleation in most cases or exenteration in some cases followed by post-operative deep X-ray therapy in either case. Three years has been taken as the critical period of survival. 35 bilateral cases could be followed up and none of them was known to have survived. 95 unilateral cases could be followed up and 18 of them are living at present. No patient with tumour in the optic nerve and no patient with extra-ocular extension are known to be living at present. Patients with massive choroidal involvement also did not survive. In all the fatal cases where the globe was not obviously involved, the optic nerve was microscopically found to be invaded by the tumour cells. In no case that recovered was the nerve found involved. In all the recurrent cases and in all cases with extraocular extensions, exenterations of the orbit were done, but all the patients were known to have died.
Five cases of retinoblastoma died in hospital. Autopsy could be done in three cases. In other cases autopsy was refused. In all three cases there were intracranial extensions via the optic nerve.
| Comment|| |
Retinoblastoma is not only the commonest intraocular tumour but is also found to be the most common tumour of the eye in the present study. In this series as in others, males were more affected than females. The disease had no particular predilection for any particular eye. Bilateral incidence in the present series was 27 per cent which is a bit on the lower side than the observation of Reese (1931) who found bilaterality in a third of all cases. The disease is said to have a strong heredity tendency. Majority of the cases are sporadic as was also found in the present series of cases. Reese (1948) reported six survivors with 10 children, nine of whom had bilateral retinoblastoma. Herm and Heath (1956) reported eight survivors with 16 children, three of whom had bilateral retinoblastoma. One of the present cases had two children, both of whom had unilateral retinoblastoma. The history of yellow reflex through the pupil was found in 93 per cent in the present observation. The interval of the appearance of yellow reflex and the beginning of the treatment varied from a few weeks to a few years. Delay in seeking treatment naturally made the prognosis worse. The disease is a congenital one, as in seven cases the history of yellow-reflex was since birth.
The tumour arises from the nuclear layers of the retina, so the tumour has a special affinity for invading the optic nerve. Once the tumour is in the optic nerve it has easy passage for intracranial extension. For this reason death from retinoblastoma by intracranial spread is common. Retinoblastoma is a very malignant disease and once it comes out from within the confines of the, eyeball it can widely disseminate and then becomes a fatal condition. Recurrent tumours and metastatic lesions did not respond to the drastic operation of exentration of the orbit followed by deep-X-ray therapy. Out of the 34 cases of retinoblastoma with metastatic lesions treated by Reese (1951) none survived. Carbajal (1958, 1959) treated intensively a number of cases of retinoblastoma with generalized metastases and all had succumbed ultimately.
It has been noted that in the survivors, the tumours were well differentiated as evidenced by the presence of good rosettes whereas in the fatalities there were few or no rosettes. In the present study extraocular extensions and optic nerve involvements were found to be more than in any other series. This can be only due to greater delay on the part of patients in seeking treatment after the beginning of symptoms. The patients who came early had good prognosis. The higher mortality of the present series of cases was therefore due to many advanced cases, more extraocular extensions and larger number of cases of optic nerve involvements. The present 18 survivors had some common features, namely, (1) no optic nerve involvement, (2) no extraocular extensions, (3) early treatment, within weeks of the discovery of a yellow pupillary reflex, (4) early enucleation followed by deep x-ray therapy and (5) good differentiation of rosettes in the histological picture. Therefore retinoblastoma patients should be treated as emergency cases. Thus it can be inferred that early diagnosis and quick and thorough treatment are the two important criteria for reducing the fatalities.
| Summary|| |
140 cases of retinoblastoma have been studied over a period of 11 years (1950-1960). 130 cases could be followed up. 18 patients are living at present. A higher mortality rate of this series is attributed to a large number of advanced cases, who reported late for treatment.
Instances of familial incidence in four families are recorded.
Optic nerve involvement and histological differentiation are discussed as signs of prognostic value.
| Acknowledgement|| |
I am grateful to Dr. P. K. Sarkar, Pathologist for valuable advices.
| References|| |
Carbajal, U. M. (1958), Amer. J. Ophthal., 43, 391.
Carbajal, U. M. (1959), Amer. J. Ophthal., 48, 47.
Herm, R. J. and Heath, P. (1956), Amer. J. Ophthal., 41, 22.
Reese, A. B. (1931), Arch. Ophth., 5, 269.
Reese, A. B. (1948), Arch. Ophth., 40, 553.
Reese, A. B. (1951), Tumours of the Eye. Hoeber. New York, p. 67.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]