Childhood Cancer

Childhood Cancer

Diagnosis and staging

After a parent or doctor notices the abnormality in a child’s eye(s), an ophthalmologist should examine the child. If retinoblastoma is suspected, the child should be referred to a major medical center with expertise in treating children with cancer. There, a pediatric oncologist and an ophthalmologist with expertise in retinoblastoma will obtain a full medical history, perform a thorough physical examination, and examine the child’s eyes under anesthesia. An ophthalmic ultrasound, a magnetic resonance imaging (MRI) scan, and/or a computerized tomography (CT) scan may be done at this time. The ophthalmologist will often use a microscope with a camera attached (called wide-field fundus photography) and an imaging technique that uses light rather than sound waves or radiation (called spectral domain optical coherence tomography) to carefully examine all areas of the retina. Once these tests are completed and retinoblastoma is diagnosed, the pediatric oncologist will work with the ophthalmologist and other specialists to formulate the best treatment plan.

I first noticed the weird reflection in Jayden’s right eye in photos when he was 9 months old. I asked the pediatrician three times about it, but he said it was nothing. Jayden was rubbing his eye a lot, and now I know he must have had a lot of pressure and it hurt. We were at his 15-month visit when the doctor said it was fine for the third time. He didn’t dilate his eye or do a close examination, or he would have seen the tumor and noticed that Jayden’s eye didn’t respond to light at all since he was already blind. One week later the swelling was so bad I took him to the urgent care, and when it was even worse the next day I drove him to the emergency room where he was finally diagnosed. The ophthalmologist told us later that given the size of the tumor, he had probably been blind since he was 7 months old. He hit all his development milestones early, and I really didn’t suspect anything was wrong. I’m in early childhood education so I know what to expect. I felt bad that I didn’t catch the fact that he couldn’t see.

A process called staging is done to find out the extent of the disease. Results from the CT or MRI are used to establish the extent of disease in both eyes, orbits (bones around the eyes), optic nerves, and the brain. If the CT or MRI shows that the disease has spread outside the eye, the pediatric oncologist may perform a lumbar puncture (spinal tap), nuclear bone scan, bone marrow biopsy, and/or bone marrow aspiration. These procedures are described in Chapter 10, Coping with Procedures.

From the beginning, we always talked to our son about what was happening, about what he might be feeling, about how sorry we were that we couldn’t avoid having him go through a specific procedure. He understood our love and concern. We always felt it was important to be honest with him about what was happening because he had a right to know. We bought him a doctor kit, a Playmobil® operating room, and kept one of his anesthesia masks for him to play with. We gave him empty dilating drop bottles so he could be the one to put the eye drops in a willing patient (stuffed animal).

Treatment options for retinoblastoma depend on the location and size of the tumor and the extent of tumor cell growth near the original tumor (called tumor seeding). Tumors may be intraocular (within the eye) or extraocular (spread beyond the eye). In North America, the International Classification for Intraocular Retinoblastoma is the most widely used method for staging retinoblastoma in children.

International Classification for Intraocular Retinoblastoma

•  Group A. Tumors with a width of 3 mm or less that are in the retina and not near the foveola (center of vision) or the optic disc

•  Group B. All tumors in the retina that don’t fit the Group A criteria.

•  Group C. Distinct local tumor(s) with minimal seeding under the retina or minimal seeding in the vitreous (the gel portion of the eye)

•  Group D. Large tumors, tumors with indefinite edges, or tumors with diffuse seeding under the retina or vitreous

•  Group E. A very large tumor that may reach to the front of the eye and cause high eye pressure (glaucoma), bleeding, or other extreme damage

Children with retinoblastoma should be followed closely by their medical team from diagnosis to age 5 to monitor for recurrence or spread of the cancer outside the eye. The frequency of these exams depends on many factors, including:

•  Whether the disease is in one or both eyes

•  The number, size, and location of tumors

•  Family history

•  The type of treatment.

After age 5, the chance of recurrence or spread is very low, but children should still be seen at least once a year by an ophthalmologist and a pediatric oncologist to monitor for late effects resulting from treatment.