About the Ocular Oncology Program
Tumors related to the eye, also known as ocular tumors, can range from harmless to potentially life-threatening and can be primary (first discovered in the eye) or metastatic (spreading from another location). Retinoblastoma is the most common type of ocular tumor found in children.
The ocular oncology service brings together experts in ophthalmology, oncology, neuro-interventional radiology, cancer genetics, and radiation oncology to provide exceptional, coordinated care to patients diagnosed with eye tumors.
We primarily diagnose and treat children with retinoblastoma at the Childhood Solid Tumor Center. Our team also has experience treating a wide range of other childhood conditions that affect the eye, including:
- Conjunctival tumor (benign or malignant)
- Choroid and retina tumor
- Congenital hypertrophy of the retinal pigment epithelium (CHRPE)
- Eyelid tumor (benign or malignant)
- Iris tumor (benign or malignant)
- Iris nevus
- Iris metastasis
- Iris cyst (congenital or acquired)
- Intraocular opportunistic infection/tumor
- Metastasis to the eye
- Optic nerve tumor (benign or malignant)
- Orbital pseudotumor (intracranial hypertension)
- Orbital tumor (benign or malignant)
- Retinal astrocytic hamartoma
- Retinal pigment epithelium (RPE) carcinoma
- Vascular tumor (hemangioma)
We also provide specialized care for certain genetic syndromes that cause tumors or other symptoms within the eye. Experts in our Pediatric Cancer Genetic Risk Program can offer patients genetic counseling and tumor surveillance.
Common syndromes that may cause eye tumors or other complications include:
- Hereditary retinoblastoma is associated with a high risk for multiple retinoblastomas in both eyes.
- Familial adenomatous polyposis (FAP) can sometimes lead to congenital hypertrophy of the retinal pigment epithelium (RPE), a pigmented lesion in the retina.
- DICER1 syndrome is associated with ciliary body medulloepithelioma (CBME), a rare ocular tumor typically found in children under 10 years old.
- Tuberous sclerosis is a genetic disorder that causes tumors throughout the body, including retinal tumors.
- Neurofibromatosis can sometimes cause tumors to develop on the optic nerve (optic glioma).
- Von Hippel-Lindau syndrome (VHL) is characterized by tumors and cysts found in different parts of the body, including the retina.
How We Diagnose and Treat Childhood Ocular Tumors
Our doctors will perform a complete eye exam under anesthesia to view and examine the entire eye. They will also order several different tests to diagnose an ocular tumor, including:
- Eye ultrasound
- Eye photographs
- Optical coherence tomography
- Fluorescein angiography
- Other advanced imaging studies
After diagnosis, your medical team will recommend a treatment plan that considers:
- The tumor's type, location, and size
- Your child's age, overall health, and medical history
- The extent of disease
- The doctor's expectations of how the tumor will behave
Treatments may include:
- Chemotherapy: We offer an innovative new form of chemotherapy known as intra-arterial chemotherapy. Doctors use it to treat certain types of retinoblastoma. The treatment involves injecting the chemotherapy directly into one of the main blood vessels of the eye. This approach minimizes the amount of contact the chemotherapy has with the rest of the body. We also use systemic chemotherapy and intravitreal chemotherapy.
- Surgery: If the cancer does not respond to other treatments, we may need to perform surgery to remove the tumor.
- Focal therapy: We use cryotherapy (cold) and thermotherapy (heat) to treat small tumors.
- Radiation therapy: Doctors may use external radiation or targeted radiation with plaque brachytherapy.
- Stem cell transplant: For the rare patient with metastatic disease, high-dose chemotherapy with stem cell transplant is an option that we can provide.
After treatment, we continue to care for your child's physical, mental, and emotional health through our pediatric survivorship programs.