OVERVEIW OF RETINOBLASTOMA PATIENTS IN NORTH-EASTERN SCENERIO


In this article, we endeavor to review demographic, clinical, classification and staging of the disease, treatment and survival of cases of retinoblastoma seen in a tertiary eye care center in the North- Eastern region, a 5( five) year study.
Background
We all know that retinoblastoma is the most common intraocular malignancy in pediatric age group. In the western literature, the incidence of retinoblastoma is 1 in 15,000 live births. In India, it is estimated to be approx 28 cases per 100,000 children (ICMR survey).The tumour is treatable if diagnosed early and promptly. However in our country, there are several factors leading to delayed diagnosis like lack of knowledge about the disease, social myth, inadequate treatment facilities, financial constraint, lack of trained doctors etc. As a results of all these ,these tumours often present as late stage at the time of diagnosis, and it is found that it has already spread outside the eye affecting the prognosis for sight and life.
Demographic profile
There are about 204 cases recorded in Sri Sankaradeva Nethralaya since 2009 to till date .Out of these, 111 are male and 93 are female patients.
We have found that most of retinoblastoma cases are occurring below 1 year of age and also these cases are mostly bilateral. As the age increases, the incidence of retinoblastoma also goes down. In our institute, most cases recorded are unilateral and only in one patient, family history of retinoblastoma is found. In this single familial retinoblastoma case, patient's own brother died of bilateral retinoblastoma.

 

Laterality

Unilateral
Bilateral
Total
155
49
204(253 eyes)

Most of the cases of RB belong to Hindu religion (50%) and also more than 90% of cases are from below poverty line. In our study, we have found that most of the cases are from village areas and suburban areas.

 

Religion

Hindu
Muslim
Christian
Other
Total
109
65
25
05
204

In the preferences of eye, it was seen that both eye affects almost equally as right eye affected in 128 eyes and left eye affected in 130 eyes.
The 204 cases of retinoblastoma, have came to our institute for treatment from various states of North-eastern states including Assam, Tripura, Manipur, Arunachal Pradesh, Nagaland and Meghalaya. Also few cases have come from our neighboring states like West Bengal and Bihar. Most of the cases are refereed by local ophthalmologists; a small number of patients came directly for enhanced treatment. The table below depicted the percentage of cases coming from different states.

 

States (162 Patients)

Assam
Arunachal Pradesh
Manipur
Mizoram
Tripura
Meghalaya
Nagaland
West Bengal
Bihar
159
10
03
05
10
11
01
03
02

 

Clinical profile

Most of the patients attending our institute came with chief complains of white papillary reflex , followed by squint, decreased vision, red eye and protruding eye (in decreasing frequency).Less common symptoms were swelling of eyelids, watering from the eye, pain , injury etc.
Symptoms

Leucocoria
Squint
Red eye
Protruding eye
Reduction in vision
Others
154
11
10
19
10
10

In the classification of tumour, we followed international classification for intraocular disease from Group A to Group E and others as extraocular tumours .We also followed Reese/Ellsworth staging for intraocular disease and clinical staging was done by Chantada classification of Retinoblastoma.

 

INTERNATIONAL CLASSIFICATION FOR INTRAOCULAR DISEASE

Group A - Very low risk
Right Eye
Left Eye
Both Eye

 

Small discrete intraretinal tumours away from the foveola and disc
All tumours are 3 mm or smaller in greatest dimension, confined to the retina and
All tumors are located further than 3 mm from the foveola and 15 mm from the optic disc

 

 

Group B - Low risk
Right Eye
Left Eye
Both Eye

All remaining discrete retinal tumors without seeding
All tumors confined to the retinal not in group A
Any tumor size and location with no vitreous or subretinal seeding

 

Group C - Moderate risk
Right Eye
Left Eye
Both Eye

Discrete local disease with minimal focal subretinal or vitreous seeding
Tumor(s) must be discrete
Subretinal fluid, present or past without gross seeding, involving up to one quadrant of retina
Local subretinal seeding, present or past less than 5 mm from the tumor
Focal the vitreous seeding close to discrete tumor

 

Group D - High risk
Right Eye
Left Eye
Both Eye

Diffuse disease with significant vitreous and/or subretinal seeding
Tumor(s) may be massive or diffuse
Subretinal fluid, present or past up to total retinal detachment
Diffuse subretinal seeding, may include subretinal plaques or tumor nodules
Diffuse or massive vitreous disease may include "greasy" seeds or avascular tumor or masses

 

Group E- Very High risk
Right Eye
Left Eye
Both Eye

 

 

INTRAOCULAR DISEASE ONLY - Reese/Ellsworth Stage:

1. Group I   Very favorable
A. Solitary tumor, < 4 disk diameters, at or behind the equator
B. Multiple tumors, none >4 disk diameters, all at or behind the equator
2. Group II   Favorable
A. Solitary tumor, 4-10 disk diameters in size, at or behind the equator
B. Multiple tumors, none n4-10 disk diameters, behind the equator Doubtful
3.Group III
Doubtful
A. Any lesion anterior to the equator
B. Solitary tumors larger than 10 disk diameters behind the equator Unfavorable
4. Group IV
Unfavorable
A. Multiple tumors, some large than 10 disk diameters
B. Any lesion extending anteriorly to the ora serrata
5. Group V
Very unfavorable
A. Tumors involving more than half the retina
B. Vitreous seeding

 

Most of the cases were diagnosed in advanced intraocular stages with secondary glaucoma or anterior segment involvement or extraocular involving orbit or optic nerve. Few cases were presenting with severely protruded eye or intracranial extension.
As per Chantada staging which depends on types of treatment given to individual patients, we followed 3 different staging - chantadta baseline, Chantada after 1st enucleation and Chantada after 2nd enucleation.

 

CLASSIFICATION OF RETINOBLASTOMA (CHANTADA ETAL PBC AUGUST 2005)( BASELINE )

 

STAGE 0. PATIENTS TREATED CONSERVATIVELY
Stage I. Eye enucleated, completely resected histologically
Stage II. Eye enucleated, microscopic residual tumor
Stage III. Regional extension
  a. Overt orbital disease &
  b. Preauricular or cervical lymph node extension)
Stage IV Metastatic disease
  a. Hematogenous metastasis (without CNS involvement)
 
  1. Single lesions
  2. Multiple lesions
  b. CNS extension (with or without any other site of regional) or Metastatic disease
 
  1. Prechiasmatic lesion
  2. CNS mass
  3. Leptomeningeal and CSF disease

 

Chantada Staging (Baseline)

Stage 0
Stage 1
Stage 2
Stage 3
Stage 4
86
50
03
52
13

 

CLASSIFICATION OF RETINOBLASTOMA (CHANTADA ETAL PBC AUGUST 2005) (AFTER THE FIRST ENUCLEATION )

STAGE 0. PATIENTS TREATED CONSERVATIVELY

Stage I. Eye enucleated, completely resected histologically
Stage II. Eye enucleated, microscopic residual tumor
Stage III. Regional extension
  a. Overt orbital disease
  b. Preauricular or cervical lymph node extension)
Stage IV Metastatic disease
  a. Hematogenous metastasis (without CNS involvement)
 
  1. Single lesions and
  2. Multiple lesions
  b. CNS extension (with or without any other site of regional) or Metastatic disease
 
  1. Prechiasmatic lesion
  2. CNS mass
  3. Leptomeningeal and CSF disease

 

Management

Before starting any treatment, a detailed history regarding birth history (gestational period, type of delivery, prenatal history, postnatal history, milestones, birth order etc), immunization history, age of onset, first sign noticed by the parent, family history of retinoblastoma or other eye cancer or any other malignancies were taken.
Regarding birth history, most of the patients were of full term with normal delivery and with no significant prenatal or postnatal abnormalities. Most of the patients were on regular immunization schedule and with regular milestones.
Most common investigation done was clinical examination, examination under anaesthesia, ultrasonography, CT scan and MRI. Frequency of different investigation were depicted in the below table. Treatment of every patient was tailored according to the grouping of the tumour done after different investigations.

 

Investigation

Enucleation
Chemotherapy
EBRT
Local
No secondary treatment
28
30
05
07
Expired
Completed treatment
Ongoing treatment
40
25
10

 

Bilateral (total 49 patients)

Primary treatment

Enucleation
Chemotherapy
No treatment (11)
06
28
Expired
Lost for follow up
Spontaneous regression
financial crisis
07
01
00
07

 

Secondary treatment (34 patients of primary treatment)

Enucleation
Chemotherapy
EBRT
Local
No Secondary treatment (15)
08
05
01
04
Expired
Ongoing Chemotharapy
14
02

 

Treatment of unilateral patients

Primary Treatment( total 155 patients)

Enucleation
Chemotherapy
EBRT
No treatment (total patients 27)
68
40
02
Expired
Spontaneous regression
Lost for follow up
financial crisis
14
06
03
22

 

Secondary Treatment (Of the primary treatment group of 110 patients.)

Enucleation
Chemotherapy
EBRT
Local treatment
No secondary treatment( total patients 49)
17
27
03
03
Expired
Completed treatment
Ongoing treatment
25
23
12

In bilateral cases, after primary treatment, enucleation was done in the eye with advanced disease and eye salvaging treatments were tried in less advanced eye. In one bilateral case, both eyes were enucleated in the same sitting to save the life of the child. But the child died after few months due to progressive disease. In 2 cases, after enucleating the more advanced diseased eye, we tried almost all eye salvaging procedures like up to 12 cycles of chemotherapy, different local treatment, even courses of EBRT. But eventually, we had to remove the other eye also. Now both children are doing fine and parent are preparing to send them to blind school for their education.
In unilateral cases, both the group with primary treatment of enucleation followed by secondary chemotherapy in high risk cases and primary chemotherapy followed by enucleation were doing fine and now on regular follow up with us.

 

Survival Status
Survival of retinoblastoma patient depends on many factors like size and location of the tumor, laterality, spreading of the tumor, associated complications like retinal detachment, glaucoma etc, family history of retinoblastoma and/or other cancer, the wishes and concerns of the parents, availability of the facilities etc.
All cases were called for check up in every 3 months and follow up was done with detailed examination of fundus regarding tumour size, sub retinal fluid, vitreous seeds, occurrence of new tumours , activity of regressed tumour, lens status etc. Detailed examination of orbit was done in post enucleated cases. Ultrasound and/or CT Scan was repeated as needed.
Patients not come for check up at given date were reminded through phones. Few patients were lost to follow up due to wrong mobile number or change of address. Social worker was sent for home visit to trace the lost cases. We also sent letters to long distance patients to know about the status of the patients.

 

Follow up cases(204 patients)

Expired
Regular
Lost for follow up
72
121
11

 

Expired patients (total 72 patients)

With treatment
Without treatment
With progressive disease or recurrence
With incomplete treatment
With complication of treatment
20
36
02
14

 

Regular follow up (Total 121patients)

Completed treatment
With progressive disease
Spontaneous regression
Ongoing treatment
With no treatment
58
17
06
20
20

 

In our North-Eastern states as most of the patients are from lower socio economic background and from village or semi urban areas, awareness about the disease is very less. And many social and ritual stigmas are also associated with the word cancer. Once child is diagnosed with eye cancer, the most common thinking is to avoid any form of treatment as cancer will eventually kill the child. After counseling the parent that there are many treatment available for this cancer and there are good chances of survival of the child with prompt and appropriate treatment, next burden is their socio economic status. Incomplete treatment is also a big problem in our scenario. One cancer patient in one family is a big burden to their both financial and mental status.
Loss for follow up is also a big concern in this region. From our experiences we found that most of the mobile numbers of the patients are PRE PAID numbers and hence they keep changing which added to all the problems of effective communications. Home visit by the social worker at times not feasible due to difficult terrains of the neighbouring states.We have been networking with different centers, NGOs and Medical Officer of neighboring states for the RB registry. Awareness about the disease amongst ophthalmologists, medical officers, health workers are the most important factors to reduce the high mortality (43.2%) and increased rate of loss for follow up cases (6.7%)found in this region. Awareness about the disease is also very necessary to diagnose these cases in early stage(most of the diagnosed in late stages, 90% cases in group D, E and extraocular) , so that we can salvage not only the eye also the vision.

Dr.(Mrs) Sumita Sarma Barthakur
Academic & Research Officer
Sri Sankaradeva Nethralaya, Guwahati

 

 

 

 



Copyright © 2016-17, Sri Sankaradeva Nethralaya
   
Powered by