Presentation Of Retinoblastoma Research Articles

Disparities in US Retinoblastoma Presentation, Management, and Local Recurrence in the National Cancer Database, 2004–2016

PurposeTo investigate patient-level differences in retinoblastoma presentation, treatments, and outcomes within the United States. DesignRetrospective registry-based analysis. Participants1,404 retinoblastoma cases in the National Cancer Database, 2004-2016, a US-based cancer registry. MethodsPatient characteristics and treatments were investigated over time. Primary treatment was classified as enucleation, local tumor destruction, chemotherapy, and/or radiation. Multivariable logistic regression models evaluated extraocular disease at presentation, treatment, and local recurrence following primary globe-sparing therapy. Main Outcome MeasuresOdds ratios for extraocular disease at presentation; primary treatment modality; local recurrence after primary globe-sparing therapy. ResultsExtraocular disease affected 13% of patients at presentation (N=178). All-cause mortality among the entire cohort was 3.1% (n=44) at last follow-up Those who were non-white, uninsured or had government-funded insurance, or with non-metropolitan residence had significantly greater odds of extraocular disease (OR 2.21-3.64 for non-white vs. white non-Hispanic patients, OR 2.05-2.95 for uninsured or Medicaid/Medicare/government-funded vs. private/commercial insurance, and OR 1.80 for non-metropolitan vs. metropolitan residence). Between 2004-2016, utilization of chemotherapy (55% to 73%) and local tumor destruction (17% to 27%) increased. Enucleations remained over-represented among Hispanic patients (63% received enucleation in 2016, vs. 35% non-Hispanic patients; OR=1.83, (95% CI 1.22-2.75) for enucleation among Hispanic vs. white, non-Hispanic patients). Patients with Medicaid/Medicare/government insurance and non-metropolitan residence also had higher odds of enucleation, and non-metropolitan patients had higher odds of local recurrence after primary globe-sparing therapy. ConclusionsDespite overall decline in enucleation and increase in globe-sparing therapy between 2004-2016, Hispanic, Medicaid/Medicare/government-insured, and non-metropolitan patients continued to have higher odds of extraocular disease at presentation and higher odds of undergoing enucleation as primary therapy. This suggests limitations in access to care and that shifts towards globe-sparing treatment (chemotherapy and local tumor destruction) did not occur equally across all patient groups. Further investigations into these disparities is warranted.

Microvascular flow ultrasound imaging for retinoblastoma

PurposeTo present the results of a pilot study of microvascular flow imaging (MFI) in characterizing tumor vasculature of retinoblastoma. MethodsThe medical records of consecutive patients with retinoblastoma presenting at our institution between July 2019 and June 2022 that were imaged using MFI were reviewed retroactively. Each patient underwent diagnostic evaluation according to standard of care by examination under anesthesia with fluorescein angiography and ocular ultrasound imaging, including color Doppler and MFI. ResultsThirteen eyes of 10 patients with retinoblastoma were included. MFI showed a prominent feeder vessel in 8 eyes, basket vasculature in 6 eyes and tumor bed vascularity in 10 eyes. MFI showed a more extensive vascular branching pattern that was not visible on color Doppler and fluorescein angiography in all eyes. ConclusionsMFI of retinoblastoma patients could add information about tumor vascularity not detectable by color Doppler or fluorescein angiography. Further study is needed to determine whether this information could be used to predict prognosis for ocular salvage and tumor response to treatment.

Presentation pattern and survival rate of retinoblastoma following chemotherapy: a prospective study

BackgroundThis study presents the clinical pattern of presentation and survival rate of retinoblastoma, which is the most prevalent form of pediatric intraocular cancer. The aim of this study is to provide baseline information about the clinical presentation and management of retinoblastoma at ECWA Eye Hospital. Additionally, the study identifies priority areas for enhancing medical care for children diagnosed with this cancer. ECWA Eye Hospital, situated in Kano State, Nigeria, is a specialized eye center located in the North-Western region of the country.MethodsA prospective study spanning five years was conducted at ECWA Eye Hospital to investigate clinically diagnosed cases of retinoblastoma. The study took place from January 2018 to December 2022. The patients received standardized pre-medication and chemotherapy protocols for retinoblastoma. Subsequently, a five-year follow-up was conducted to monitor the patients’ progress. The collected data was analyzed, descriptive statistics were generated, and the survival rate was calculated.ResultsDuring the five-year study period, a total of 35 cases of retinoblastoma were diagnosed. The patients had an average age of 3.21 ± 1.32 years. The most common presentation patterns observed were fungating ocular mass and proptosis. Among the cases, there were 10 instances of bilateral proptosis and 25 instances of unilateral proptosis. While no patients exhibited bilateral leukocoria, eight cases of unilateral leukocoria with anterior segment seedlings were identified. The additional patterns of presentation are proptosis, leukocoria, fungating orbital mass, redness and loss of vision. The mortality rate was 80% (28 cases), while the survival rate was 20% (7 cases). Notably, all the survivors had unilateral retinoblastoma.ConclusionThe majority of cases observed at ECWA Eye Hospital involve advanced retinoblastoma. In low-resource settings where alternative treatment options are limited, chemotherapy is considered a viable treatment option. Early presentation of retinoblastoma in patients may lead to a higher survival rate when chemotherapy is administered.

Retinoblastoma associated with total exudative retinal detachment: Treatment and outcomes.

To study the clinical presentation, treatment, and outcomes of eyes presenting with retinoblastoma (RB) and total retinal detachment (RD). Retrospective study of 50 eyes in 43 patients. Mean age at presentation was 15 months (median, 9 months). RB associated total RD was unilateral (n=36; 84%) or bilateral (n=7; 16%). The mean tumor basal diameter was 17 mm (median, 18 mm) and the mean tumor thickness was 13 mm (median, 12 mm). Associated features included subretinal seeds (n=26; 52%), vitreous seeds (n=13; 26%), and subretinal exudation in (n=4; 8%). Based on 8th edition of American Joint Committee on Cancer classification, the tumors were classified as cT2a (n=19; 38%), cT2b (n=28; 56%), or cT3c (n=3; 6%). Primary treatment included intravenous (n=48; 96%) or intra-arterial chemotherapy (n=2; 4%). Over a mean follow-up period of 30 months (median, 29 months; range, 7 to 62 months), RD resolved in 41 (82%) eyes, tumor recurrence was noted in 39 (78%) eyes, globe salvage was achieved in 35 (70%) eyes and one (2%) patient died due to metastasis. Relatively high tumor recurrence rate is noted in RB presenting with total RD. With appropriate treatment, 70% of the globes can be salvaged.

Causes of delayed presentation of retinoblastoma

Aim: The aim of the study was to determine the Causes of Delayed Presentation of Retinoblastoma. Materials and methods: This study was carried out at the department of ophthalmology DHQ TH Bannu from January 2022 to January 2023. In this research newly recruited patients were included who were receiving therapy for retinoblastoma during that time. For this study, parents of patients who came into the hospital were gathered. A form via the patient's information was noted. First symptom, duration after onset of initial symptom, gender, age at first visit. The data analysis software used was SPSS version 20. The importance of each factor was determined using the Pearson Chi Square test. Results A total of 80 participants were include in the study. 52(65%) patients had delayed presentation, 57(71%) had delayed referral presentation and 65(81%) had advanced diseases at the time of presentation. Various factors involved in the delay presentation. These factors are, living in a remote region; financial hardship; social pressure; knowledge of the severity of the illness; lack of transportation; Fear of enucleation and looking for alternative medications or non-medical treatments. Each factor significance was calculated through chi-square test.

Presentation and management outcomes of Retinoblastoma among Syrian refugees in Jordan.

The humanitarian crisis in Syria has had a profound impact on the entire region. In this study, we report the patterns of presentation and management outcomes of Syrian patients with Retinoblastoma (Rb) treated at a single tertiary cancer center in Jordan. This is a retrospective comparative study of Syrian refugees and Jordanian citizens who had Rb between 2011 and 2020. Collected data included patient demographics, presentation, tumor stage, treatment modalities, eye salvage rate, metastasis, and mortality. Thirty Syrian refugees (16 (53%) had bilateral disease) and 124 Jordanian citizens (51(41%) had bilateral disease) were diagnosed with Rb during this period. The median age at diagnosis for refugees was 10 and 32 months for patients with bilateral and unilateral Rb consecutively, compared to 6 and 28 months for citizens. The median lag time between signs of disease and initiation of treatment was 3 months for refugees, compared to 1 month for citizens.Refugees were more likely to present with a more advanced stage (p=0.046). Out of 46 affected eyes in refugees; 32 (70%) eyes were group D or E, while out of 175 affected eyes among citizens; 98 (56%) eyes were group D or E. Therefore, refugees with Rb were more likely to mandate primary enucleation (48%) compared to citizens (25%) (p=0.003). However, out of 24 eyes among refugees who received conservative therapy, 15 (62%) eyes were successfully salvaged, while out of 131 affected eyes among citizens who received conservative therapy, 105 (80%) eyes were successfully salvaged (p=0.06). Two (7%) of the refugees and four (3.2%) of the citizens with Rb died from metastasis. Syrian refugees with Rb presented with more advanced disease due to delay in diagnosis and referral that increased the treatment burden by decreasing the chance for eye globe salvage. However, patients who received the timely intervention had a similar outcome to citizens with Rb; probably a reflection of the management of all patients at a single specialized center. We advocate for the timely referral of refugees with this rare life-threatening tumor to a specialized cancer center for the best possible outcome.

Imaging Characteristics of Retinoblastoma: A Single Tertiary Care Center Experience from Pakistan

Background: Retinoblastoma is a rare tumor of eye in the pediatric population. It is seen in every 15000-18000 births with no gender or racial predilection. Retinoblastoma can have a variable presentation ranging from leukocoria to a mass in the eye. Aim: To provide physicians a snapshot of different modes of presentation. This study employs the data from computed tomography (CT) and magnetic resonance imaging (MRI) and analyzing it for frequency of different presentations from a radiological point of view. Methods: This was a retrospective cross-sectional study carried out at the National Institute of Child Health (NICH) which is the largest tertiary care pediatric center in Karachi, Pakistan. Records of children from 1 month to 16 years who were diagnosed with retinoblastoma between March 2021 to March 2022 were accessed from the database and assessed. The collected data was then analyzed on SPSS 26. Results: The most frequent presentation noted was leukocoria followed by vision loss, swelling, pain and redness. Calcification was the most frequently reported feature in CT scan whereas retroglobular involvement was mostly seen in MRI scans. Chi square showed a statistically significant correlation between age at presentation with presenting symptoms (p=0.04) and sellar involvement (p=0.00). Additionally, statically significant association was seen between family history and laterality in our study. Conclusion: MRI is the recommended modality due to its sensitivity and superior soft-tissue resolution. CT scan is superior to MRI in detecting calcifications. In developing countries due to a lack of proper facilities and awareness, there is a disparity in the prognosis and presentation of retinoblastoma. Bilateral tumors are most commonly heritable and patients presenting with tumors in both eyes are recommended a through genetic workup. Keywords: Retinoblastoma, ocular tumor, leukocoria, red eye, Magnetic resonance imaging, computed tomography

Seasonal Variation in the Diagnosis of Retinoblastoma

ABSTRACT Purpose To determine the seasonal variation in the diagnosis of retinoblastoma in a global sample of children and to investigate predictors of seasonal trends. Methods Data were collected through a global, multicenter, 1-year cross-sectional analysis that included all treatment- naïve retinoblastoma patients presenting to participating centers between January 1, 2017, and December 31, 2017. Due to variations in days per month, data were normalized to a 30-day/month calendar. Observed data were compared to a simulation study of expected results using a uniform distribution. Results Patient-level data were available for 4,351 children from 276 centers in 153 countries, of which 3,966 had a month of presentation recorded. There were relative peaks in disease presentation in January and July, with lower counts in November and December (p = .0015). No covariates were found to be significantly associated with the seasonal trend. Two covariates, patient age at presentation and extraocular tumor spread, showed a moderate association with month of presentation. Conclusion Our findings suggest seasonal trends in the presentation of retinoblastoma across the world. However, these trends do not appear to be related to income level of a country, climate, or other covariates. Any connection between seasonal variation of retinoblastoma presentation and retinoblastoma outcomes remains unclear or not present.

Epidemiological and Clinical Presentation of Retinoblastoma among Nepalese Children in 2019.

Suresh B.K. RasailyObjectives Retinoblastoma (RB) is rare but potentially fatal if left untreated. This study aimed to evaluate the epidemiological profile, clinical manifestation, classification, and affordability of RB treatment among Nepalese children in the year 2019. Materials and Method A multicentric, multiethnic hospital-based cross-sectional study after ethical approval from the National Health Research Council was conducted from January 2019 to December 2019 by incorporating ophthalmologists all over the nation. Twenty-seven RB centers were selected. All the RB presented in the RB centers either newly diagnosed or ongoing treatment consented to the study were included and failed to consent for the study, RB survivors were excluded from the study. Data based on demographic profile, clinical manifestation, ethnical and geographical distribution, and treatment received were collected in the customized Google Form. Each case was classified at the time of diagnosis as per the International Classification of Retinoblastoma groups and different treatment modalities offered as per grouping and staging. The affordability of treatment was calculated using a catastrophic approach. Statistical Analysis Data were entered into Microsoft Excel 2010 and analyzed using Statistical Package for Social Sciences version 20. Result A total of 34 RB cases, 21 (61.76%) in ongoing treatment group and 13 (38.2%) in newly diagnosed group presented in the RB centers. Out of total, 32 (64.7%) had unilateral and 12 (35.3%) cases had bilateral involvement. The majority of patients was from Province 1 (35.3%) and belonged to the upper caste (38.2%). Leukocoria was the most common presentation (73.9%) followed by proptosis, red eye, and phthisis bulbi. More than 75% patients presented at advanced group D (54%) and E (21%) and stage 0 (90%). More than 90% of patients received systemic chemotherapy, and 42.6% received transpupillary thermotherapy. The average cost of RB treatment was estimated to be 521% of the nonfood expense of the family which is unaffordable to almost all cases (100%). Conclusion Leukocoria is the most common mode of clinical presentation in both unilateral and bilateral RBs. Early diagnosis and appropriate treatment are key to success for saving life, sight, and eye. However, community awareness programs against RB, active referral networks, and the establishment of chemotherapy centers with trained human resources are needed to reduce loss of life, sight, and eye.

Recognizing pediatric eye problems that can save lives- cases not to miss!

As pediatric ophthalmologists we deal with many conditions that present as the first manifestation of a systemic disease. Some of these cases can actually be life threatening if not diagnosed and treated in a timely manner. Physicians need to avoid the clinical decision making error of confirmation bias. The purpose of this workshop is to show and discuss cases with life saving impact that should not be missed, including eye findings that can lead to a diagnoses of occult Hirschprung disease, rare manifestations of occult child abuse, unusual presentation of retinoblastoma and others.

Clinicopathological Presentation of Retinoblastoma in Ethiopia

Introduction: The presenting symptoms of retinoblastoma in Ethiopia, as well as their relationship to the stage of the disease, are poorly understood, but they could be important as background knowledge for creating early detection initiatives. This study aimed to describe the clinical and histopathological characteristics of retinoblastoma among Ethiopian patients. Methods: A hospital-based cross-sectional analysis of all children with newly diagnosed retinoblastoma during the study period of May 2015 to December 2019, inclusive, who presented to a tertiary referral center during were included. Demographic, clinical, and histological characteristics were collected and descriptive statistics were done using SPSS Version 20.0 software. Results: A total of 217 patients (unilateral 84.3%, bilateral 15.7%) were studied. The median age at presentation was 29 months (34 months vs. 19.5 months in unilateral and bilateral cases, respectively, p < 0.001). Among all patients, 57.8% were male. Leukocoria was the most common presenting sign (37.3%) and followed by fungating mass (31.8%). Tumors were extraocular in 40.1% of cases. From the intraocular cases, 73.3% were advanced International Intraocular Retinoblastoma Classification Group D or E disease. Sixty-two (54.4%) of the eyes had high-risk pathological features. The median lag time was significantly shorter for intraocular versus extraocular cases (5 months vs. 12 months, respectively, p < 0.001). Conclusion: Our results show that the majority of Ethiopian children with retinoblastoma have delayed presentation and late stage at diagnosis. This suggests that national health promotion campaigns to increase public knowledge on the presenting signs of retinoblastoma may be critical to achieving early diagnosis. Furthermore, the development of standard management guidelines informed by this study will be helpful in managing the complex and advanced cases currently observed.

Retinoblastoma Masquerading as Spontaneous Hyphaema in a Nigeria Infant

Retinoblastoma is the most common intraocular malignancy of children and occurs mostly within the first 3years of life with an average onset at about 18months of age. The most common form of presentation is leukocoria, other less common forms of presentation includes strabismus, uveitis, proptosis, orbital cellulitis with hyphaema being one of the least forms of presentation. This is a case report of Retinoblastoma presenting with hyphaema in a Nigerian Infant and it is to bring to fore that even though hyphaema is not a common presentation in patients with Retinoblastoma, It should still not be overlooked in order to avoid the poor prognosis that is often associated with late diagnosis.

Causes of Delayed Presentation of Retinoblastoma in a Tertiary Care Hospital of Pakistan

Purpose: To find out the causes of delayed presentation of Retinoblastoma in a tertiary care hospital. Study Design: Questionnaire based Survey. Place and Duration of Study: Pediatric ophthalmology department of The Children’s Hospital, Lahore, from January 2018 to December 2019. Methods: Mothers of patients who presented in the hospital were recruited for this survey. A questionnaire with details of the patient were noted. It included first symptom, time lapse since onset of first symptom, gender, age at presentation to first doctor (general practitioner), time lapse to presentation at tertiary care center (The Children’s Hospital &amp; Institute of Child health), gender and laterality. General physical Examination was done by an oncologist and examination under anesthesia was done by a pediatric ophthalmologist. Data was analyzed using SPSS version 20. Pearson Chi Square test was used to find significance of each cause. Results: Seventy participants were included in the study. Delayed presentation was seen in 42 patients (60%). The various factors identified for delay in diagnosis and treatment in descending order are residence in rural areas 54 %, lack of finances 64%, social pressure 27%, awareness about disease severity 60%, lack of transport 54%, Fear of enucleation 38% and seeking non-medical treatment/ alternate medication 32%. Significance of each factor was calculated using chi-square test. Conclusion: The causes identified can be highlighted on national level for development of health facilities in rural areas. Key Words: Retinoblastoma, Cancer, Malignant tumor, Leucocoria.

Retinoblastoma outcomes: a global perspective

Retinoblastoma outcomes: a global perspective

Scenario of Retinoblastoma Among Bangladeshi Children – A Single Center Experience of 10 Years

Background: Retinoblastoma is the most common intraocular tumor of childhood and most detected cases are from Indian subcontinent. Here we explore the presentation of retinoblastoma with its histopathological features and treatment outcome in a tertiary eye care center in Bangladesh. Method: This was a retrospective study of 70 eyes of 60 patients in a ten years period from 2006 to 2016 who had met the criteria regarding demographic profile, clinical presentation, management and histopathology. Tumor was classified based on IIRC (International Intraocular Retinoblastoma Classification) for intraocular tumor and IRSS (International Retinoblastoma Staging System) for extraocular tumor by reviewing the data revealed from EUA, imaging and histopathology. Result: The mean age of presentation was (Table 1) 31.3 ± 21.68 months, range was 4 months to 96 months and the most frequent presenting age was 12 months. Fifty three percent of children presented between 13 months and 59 months. Mild male predominance was 53.3% (n = 32). Unilateral cases were 71.3% (n = 43) and 6.7% (n = 4) of patients had positive family history. The most common presenting sign was leucocoria (85%) followed by strabismus (18.3%) and proptosis (13.3%). Fifty five (91.6%) children presented with intraocular tumor and 64.9% were with Group E. Enucleation (91.6%) was the prime treatment modality and histopathological risk factor was positive in 51.7% cases among 63.3% cases of primary enucleation. Fifty percent of treated children were in regular follow up. Conclusion: Most children presented delayed and prime treatment modality was enucleation. Only half of the patients were in regular follow up. Generation of awareness, proper referral, information regarding treatment availability and early detection of cases can increase the survival rate and globe salvage.

Community Screening and Community Participation Along with Hospital Based Multidisciplinary Treatment Can be the Key Factors for Both Early Detection, Life and Globe Salvage of Retinoblastoma Patients in Developing Countries - Our Experience

Each year nearly 8000 new cases of retinoblastoma are detected worldwide. Revolutionary management strategy increased the survival rate of retinoblastoma above 95% but still it is a deadly cancer worldwide. Survival from retinoblastoma depends on income &gt;90% vs 40% (in high to low income countries) and metastases is higher in low-income countries. Forty three percent of global burden lives in 6 countries of Asia. An estimated worldwide death rate is more than 40% and most from Asia and Africa. Bangladesh is one of the developing countries of the South- East Asia region and retinoblastoma constitutes 83% of all pediatric cancer bellow 4 years age group. Early detection of cases and proper referral may decrease the advance disease which may achieved by strong awareness program. To save life and vision multidisciplinary team approach is a time demand now. Chittagong Eye Infirmary is a tertiary eye care center is serving retinoblastoma patients with a multidisciplinary team approach from 2017 and set up chemotherapy facilities in ophthalmic institute. This institute also started screening and local community training program to detect the disease easily and upgraded treatment facility from 2019.From January 2017 to June 2021 total 284 retinoblastoma patients were diagnosed, 104 received chemotherapy and 53 underwent enucleation with long optic nerve. Two hundred fifty six children were screened, 169 primary school teacher and 408 health worker were trained about key sign of retinoblastoma from July 2019 to February 2021. This effort was able to create awareness which showed early presentation of retinoblastoma and seeking treatment of white pupillary reflex than previous period. The hospitals also take care for visual and psycho-social rehabilitation of the retinoblastoma survivals.

Participation in community screening and hospital-basedmultidisciplinary treatment for early detection and lifeand globe salvage of patients with retinoblastomain developing countries: Authors’ experiences

Each year nearly 8000 new cases of retinoblastoma are detected worldwide. Revolutionary management strategy increased the survival rate of retinoblastoma above 95% but still it is a deadly cancer worldwide. Survival from retinoblastoma depends on income &gt;90% vs 40% (in high to low income countries) and metastases is higher in low-income countries. Forty three percent of global burden lives in 6 countries of Asia. An estimated worldwide death rate is more than 40% and most from Asia and Africa. Bangladesh is one of the developing countries of the South- East Asia region and retinoblastoma constitutes 83% of all pediatric cancer bellow 4 years age group. Early detection of cases and proper referral may decrease the advance disease which may achieved by strong awareness program. To save life and vision multidisciplinary team approach is a time demand now. Chittagong Eye Infirmary is a tertiary eye care center is serving retinoblastoma patients with a multidisciplinary team approach from 2017 and set up chemotherapy facilities in ophthalmic institute. This institute also started screening and local community training program to detect the disease easily and upgraded treatment facility from 2019. From January 2017 to June 2021 total 284 retinoblastoma patients were diagnosed, 104 received chemotherapy and 53 underwent enucleation with long optic nerve. Two hundred fifty six children were screened, 169 primary school teacher and 408 health worker were trained about key sign of retinoblastoma from July 2019 to February 2021. This effort was able to create awareness which showed early presentation of retinoblastoma and seeking treatment of white pupillary reflex than previous period. The hospitals also take care for visual and psycho-social rehabilitation of the retinoblastoma survivals.

Pattern of Presentation of Retinoblastoma in North India: A Teaching Hospital Survey

Pattern of Presentation of Retinoblastoma in North India: A Teaching Hospital Survey

Scenario of Retinoblastoma Among Bangladeshi Children – A Single Center Experience of 10 Years

Background: Retinoblastoma is the most common intraocular tumor of childhood and most detected cases are from Indian subcontinent. Here we explore the presentation of retinoblastoma with its histopathological features and treatment outcome in a tertiary eye care center in Bangladesh. Method: This was a retrospective study of 70 eyes of 60 patients in a ten years period from 2006 to 2016 who had met the criteria regarding demographic profile, clinical presentation, management and histopathology. Tumor was classified based on IIRC (International Intraocular Retinoblastoma Classification) for intraocular tumor and IRSS (International Retinoblastoma Staging System) for extraocular tumor by reviewing the data revealed from EUA, imaging and histopathology. Result: The mean age of presentation was (Table 1) 31.3 ± 21.68 months, range was 4 months to 96 months and the most frequent presenting age was 12 months. Fifty three percent of children presented between 13 months and 59 months. Mild male predominance was 53.3% (n = 32). Unilateral cases were 71.3% (n = 43) and 6.7% (n = 4) of patients had positive family history. The most common presenting sign was leucocoria (85%) followed by strabismus (18.3%) and proptosis (13.3%). Fifty five (91.6%) children presented with intraocular tumor and 64.9% were with Group E. Enucleation (91.6%) was the prime treatment modality and histopathological risk factor was positive in 51.7% cases among 63.3% cases of primary enucleation. Fifty percent of treated children were in regular follow up. Conclusion: Most children presented delayed and prime treatment modality was enucleation. Only half of the patients were in regular follow up. Generation of awareness, proper referral, information regarding treatment availability and early detection of cases can increase the survival rate and globe salvage.

Clinical picture and grouping of retinoblastoma at first presentation to a tertiary Hospital in Dar es Salaam: Implications for treatment outcomes

Objectives: To assess the clinical picture and grouping of retinoblastoma at first presentation to the oncology ward at Muhimbili National Hospital. Methods: A cross sectional descriptive hospital based study was conducted from April to December 2018. Seventy two patients who presented for the first time to Muhimbili National Hospital (MNH) with a diagnosis of retinoblastoma were consecutively sampled and recruited in the study. Visual acuity, horizontal corneal diameter, intraocular pressure, anterior and posterior segments of the eye were assessed and each eye was classified according to the International Classification of Retinoblastoma (ICRB) group. Ultrasonography, neuroimaging and histology were performed. Results: A total of 72 patients comprising of 90 affected eyes were recruited and analyzed: 39(54.2%) males and 33(45.8%) females. Majority of patients were residents of the Coastal and Lake Zones. Family history of Retinoblastoma was positive in only one patient. Fifty four (75.0%) patients had unilateral disease. Leukocoria (77.8%) and proptosis (41.7%) were the commonest first clinical signs noted by the family and health workers at MNH respectively. At MNH, both bilateral and unilateral cases presented with advanced disease of group E and extra ocular extension where 84(93.3%) eyes were indicated for enucleation. The median lag time from disease onset to presentation at MNH was 4 months. Conclusion: Leukocoria was the commonest first sign of retinoblastoma presentation at home. However, despite early presentation to primary health facilities, most patients presented to the tertiary centre of MNH late with advanced disease. Efforts to raise awareness on retinoblastoma to both health workers and the community are important for early case detection, referral, diagnosis and treatment in order to improve visual outcomes and survival rates of retinoblastoma patients.