Retinoblastoma Flashcards
What is retinoblastoma?
Malignant tumor of embryonic neural retina.
Age range for RtB?
<5 years
Pathophysiology of RtB?
Intraocular growth, prior to invasion of structures within the globe or spread to metastatic sites.
Symptoms of RtB?
Decreased visual acuity
Inflammation
Signs of RtB?
Leukocoria
Strabismus
Hyphema
Virtreous hemorrhage- black pupil
Proptosis
How do you classify RtB?
- laterality: unilateral( 2 yrs)
Bilateral (1 year)
Trilateral ( <5 years) - Focality: Unifocal / multifocal
- Genetics: hereditary - 40%
non hereditary - 60%
What is trilateral RtB?
Consists of bilateral hereditary + intracranial neuroblastic tumor of the pineal gland
Occurs in 5- 15 % of children with familial, multifocal or bilateral RtB.
How do I diagnose RtB?
Elicit red relex
Siblings of patients should be screened at regular interval at least through age 3
Siblings of patients should be screened for mutations in RB1 genetic
CT or MRI of brain and orbit
No surgical biopsy is performed
How do I investigate?
FBC
BUE and Creatinine
Lumbar puncture if there’s suspicion of CNS disease
Bone marrow biopsy in abnormal blood counts
Histopathologic evaluation if enucleation is performed.
Treatment modalities?
Systemic chemotherapy
External beam radiotherapy
Local ophthalmic therapy ( laser Ry)
Intra arterial chemotherapy, using and interventional neuro-radiology approach
Enucleation
Secondary malignacies
Osteocarcoma
Soft tissue sarcomas
Melanoma
Leukemia
Lymphoma
Breast cancer