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Establishment and Propagation of Human Retinoblastoma Tumors in Immune Deficient Mice
Published on: August 4, 2011
Retinoblastoma treated in infants in the first six months of life
Insights
Early diagnosis of retinoblastoma (a childhood eye cancer) in infants does not always mean the disease is caught early. Many infants still required enucleation or developed metastatic disease, indicating advanced stages at presentation.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Cancer Genetics
Background:
- Retinoblastoma is the most common primary intraocular malignancy in children.
- Early diagnosis and treatment are crucial for preserving vision and preventing metastasis.
- Infants diagnosed within the first six months of life are a specific focus for understanding disease progression.
Purpose of the Study:
- To evaluate the outcomes of retinoblastoma diagnosed and treated in infants within the first six months of life.
- To assess the correlation between early diagnosis age and disease stage, treatment response, and long-term prognosis.
- To identify factors contributing to treatment failure and enucleation in this young patient cohort.
Main Methods:
- Retrospective analysis of 158 children diagnosed with retinoblastoma within the first six months of life.
- Data collection included age at diagnosis, tumor staging (Reese-Ellsworth classification), laterality, treatment modalities, and outcomes.
- Mean follow-up duration was 7.4 years.
Main Results:
- The mean age at diagnosis was 3.6 months, with 68% presenting with group V tumors.
- 68% of patients had bilateral retinoblastoma, often asymmetric.
- Despite early treatment, 25% of treated eyes required enucleation, and 10 patients developed metastatic retinoblastoma and died.
Conclusions:
- Diagnosing retinoblastoma in the first six months of life does not guarantee early disease detection.
- A significant proportion of infants present with advanced disease, necessitating aggressive treatment and leading to poor outcomes.
- Further research is needed to improve early detection and treatment strategies for retinoblastoma in infants.
Abstract:
One hundred fifty-eight children with retinoblastoma received diagnoses and were treated during the first six months of life and then were observed for a mean of 7.4 years. The mean age at diagnosis was 3.6 months. Four patients received diagnoses at birth, one of metastatic disease. Ninety-four patients (60%) had leukokoria, reflecting the fact that 108 (68%) were initially seen with group V tumors (Reese-Ellsworth staging) despite their young age. One hundred seven (68%) were initially seen with bilateral retinoblastoma and 51 (32%) were seen with unilateral disease; in ten of the latter group, tumors later developed in the fellow eye. The eyes of 90 patients with bilateral involvement were asymmetric at first visit (that is, their eyes were involved at different stages of disease). Of 52 eyes receiving bilateral irradiation, 26 (50%) required further treatment, 23 (70%) of these for a resistant tumor and ten (30%) for new tumors. Despite the early age at diagnosis and treatment, 13 (25%) of treated eyes ultimately required enucleation. Metastatic retinoblastoma developed in ten patients and they died. Second, nonocular tumors developed in 13 patients. Early age at diagnosis does not ensure that the disease is caught at an early stage.
