Dr. Fairooz PM,F13955, Dr. Santosh G Honavar, Dr.Vijay Anand P Reddy
Abstract
Purpose:
To report demographics, management, and outcome of retinoblastoma in the India
Methods:
Consecutive case series of 1,543 eyes of 1,067 patients, managed over 20 years
Results:
Overall, 60% of children were <3 years of age and 45% percent were bilateral. Symptoms were leucocoria (36%), vision loss (10%), squint (7%), red painful eye (7%) proptosis (6%) and orbital cellulitis (2%). The tumor was intraocular in 94% and orbital in 6%. Protocol-based management included enucleation (52%), chemoreduction (26%), focal therapy (11%), and radiation (5%). Fifty-two percent of enucleated eyes had histopathological risk factors for metastasis and received adjuvant chemotherapy. Chemoreduction and focal therapy resulted in eye and vision salvage in 90%. Overall disease-free survival was 94%.
Conclusions:
Protocol-based management of retinoblastoma by a multidisciplinary team provides excellent prognosis for survival, eye salvage and vision salvage even in the setting of a developing country.
Introduction:
Retinoblastoma (RB) is most common intraocular tumor affecting children younger than 3 years of age often. It accounts for 3% of childhood malignancy. Cancer registries like the North American Survival Epidemiology and End Results (SEER), the European Automatic Childhood Cancer Information System and others have statistically tabulated the incidence and survival of children with retinoblastoma. From the epidemiological studies it is estimated that incidence of RB is highest in China and India. However, information from the developing countries on the incidence and survival is scanty. India has one of the highest incidences of retinoblastoma but organized data is scanty. We present a large series of patients from a multidisciplinary retinoblastoma treatment center based in an eye hospital.
Methods:
The charts of all consecutive patients diagnosed with RB between January 1988 to December 2011 at a tertiary care center in Southern India with minimum 3 years follow up was retrospectively analyzed. The data included patient’s age at presentation, gender, family history, initial symptoms, prior diagnosis and, laterality. The tumor type (endophytic, exophytic, diffuse, mixed) and classification as evaluated clinically at the time of initial examination under anesthesia was recorded. The treatment modalities (systemic chemotherapy/ chemoreduction, enucleation, cryotherapy, laser therapy, plaque brachytherapy and external beam radiotherapy) were recorded. Histopathological details in eyes that were enucleated were also recorded and analyzed. Final follow up status was also noted.
Results
There were 1543 eyes of 1067 patients included in the study who were diagnosed of retinoblastoma between January 1988 to December 2011. Five hundred and ninety one eyes were unilateral and 476 were bilateral. Majority of patients belonged to the age group of ≤36 months. The disease was asymptomatic in 9% patients. Most common symptoms were leucocoria in 36%. Others were squint (11%), visual loss (10%), red painful eye (7%), proptosis (6%), orbital cellulitis (2%) and,fungating mass (3%). Intraocular (94%) and extraocular (6%) presentation was noted. Of the 1543 eyes, tumor morphology could be analyzed only in 948 eyes. There were endophytic (67%), exophytic (12%), mixed (15%) and diffuse infiltrative (5%). Only 1055 eyes were available for the analysis of classification under International Classification of Intraocular Retinoblastoma system. Majority was classified under Group E (42%) followed by Group D (27%). Primary treatment with systemic chemotherapy (chemoreduction) alone was performed in 375 eyes and eye salvage was 90%. Enucleation was performed in 547 eyes, as a primary (n=403) and, secondary (n=144) modality. Of which histopathological risk factors were present in 152 eyes (52%). Thirty eyes with primary orbital retinoblastoma without systemic metastasis at the time of presentation whounderwent the intensive multimodal treatment protocol (neoadjuvant chemotherapy, enucleation, radiation and adjuvant chemotherapy) had 94% disease free survival. Overall survival inpatients undergoing protocol based management was 95%. Eye salvage was 65%, although chemoreduction had 90% eye salvage.
Discussion
Retinoblastoma has >98% survival and almost 100% eye salvage if detected early. Protocol based management improved vision, eye and life salvage. Our analysis shows that majority of patients present at advanced stage of intraocular RB. Extraocular presentation is not uncommon. There is a need of improving the awareness of the disease to detect early and improve vision, eye and or life salvage. Current management with introduction of systemic chemotherapy has drastically improved the outcome. Our patients received cost effective treatment, and this helped to reduce treatment abandonment. However majority of patients underwent enucleation due to advanced disease. Histopathological risk factors (HRF) were analyzed in all patients undergoing enucleation. It was found that 52% eyes had HRF and received adjuvant chemotherapy. Those patients with orbital retinoblastoma underwent intensive multimodal treatment protocol and the survival in patients who completed treatment was 94% with primary orbital RB. Thereby it is found that cost-effective protocol based management in developing countries can improve vision, eye and life salvage.
Conclusion:
Retinoblastoma burden in India is huge compared to developed countries and it is essential to instill awareness among the public and physicians for early detection for improved outcome. Strict protocol based management improves the overall outcome in RB.