Retinoblastoma Follow-up

Updated: Jul 12, 2016
  • Author: Marichelle Aventura Isidro, MD; Chief Editor: Hampton Roy, Sr, MD  more...
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Follow-up

Further Outpatient Care

Patients with treated retinoblastoma as well as siblings who are at risk of inheriting the tumor need to be monitored indefinitely.

Patients and siblings of patients in whom the risk of retinoblastoma cannot be ruled out by genetic studies should be monitored with examination under anesthesia every 3-4 months until age 3-4 years, after which they are examined under anesthesia every 6 months until age 5-6 years and then annually thereafter. At about age 8 years, most patients are able to tolerate a dilated fundus examination in the office without anesthesia and can be examined annually in the office thereafter.

Visual acuity, alignment, and general ocular health should be should be periodically examined in office. The patient and parents should be questioned about and warned about signs of secondary nonocular tumors during these examinations.

Formal examination under general anesthesia is completed 6 months after completion of radiation therapy.

For classic regression patterns, see the image below.

Classic regression patterns of retinoblastoma Classic regression patterns of retinoblastoma

As long as the tumor is not enlarging, it can be considered to be locally controlled by radiation therapy.

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Further Inpatient Care

Inpatient care is mostly supportive during the period of recuperation after surgery or during chemotherapy.

Daily attention to the cleansing and dressing of a postenucleated eye or postexenterated orbit is necessary.

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Inpatient & Outpatient Medications

Only supportive medications during chemotherapy or after surgery are needed. These include antinausea agents, broad-spectrum antibiotics, and painkillers.

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Deterrence/Prevention

Frequent ophthalmologic examination is indicated for children at elevated risk.

Estimation of risk can be completed using molecular genetics.

DNA testing can be a cost-effective component of the care of patients with retinoblastoma and their relatives.

Diagnosing the tumor as early as possible is important to prevent progression leading to metastasis and ultimately death.

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Complications

Secondary nonocular tumors can develop in survivors of retinoblastoma. In order of decreasing frequency, they are as follows: osteosarcoma, various soft tissue sarcomas, malignant melanoma, various carcinomas, leukemia and lymphoma, and various brain tumors. (See Special Concerns.)

Cataract formation: Radiation doses of 800 cGy to the lens using dose rates of 150-300 cGy/min usually lead to cataract formation in 18 months to 3+ years.

Vascular complications: Retinal vascular damage and hemorrhage may be seen after external beam radiation using 70-75 Gy with 200-350 cGy per fraction.

Bone, dental, and soft tissue effects: Hypoplasia of bone and soft tissue structures after treatment with radiation doses exceeding 3500 cGy may occur. The maxillary molar tooth buds located high in the maxilla just inferior to the posterior apex of the orbit may become irradiated with treatment. Numerous reports of failure of tooth eruption have been noted in patients with retinoblastoma treated with irradiation.

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Prognosis

In a study of 69 adult survivors of retinoblastoma assessed at an average of 31 years after diagnosis, Brinkman et al found that subjects performed within normative expectations on various measures of verbal intelligence, attention, memory, processing speed, and executive functioning; they performed above expectations in nonverbal reasoning abilities and ability to learn new information and below expectations in fine motor dexterity. [1, 2] Survivors reported more problems with working memory and task completion than adults of similar age did.

Patients who had had bilateral disease performed significantly better than those with unilateral disease on measures of verbal learning and short- and long-term verbal memory. [2] Diagnosis at less than 1 year of age was associated with better performance on a number of verbal domains, Total brain radiation exposure was negatively correlated with performance on measures of verbal learning and memory.

The prognosis in retinoblastoma is good where prompt medical care is available. The overall survival rate of retinoblastoma in the United States and Great Britain is presently greater than 85%.

The cure rate is almost 90% if the optic nerve is not involved and enucleation is performed before the tumor passes through the lamina cribrosa.

Survival rates decrease to 60% if the tumor extends beyond the lamina cribrosa even if the cut end of the nerve is free of tumor cells.

Survival rates decrease to less than 20% if the tumor cells are found at the surgical transection sight.

Death occurs secondary to intracranial extension. Treatment with EBRT results in an 85% cure rate.

Visual preservation occurs in 90% of children with group I and II disease (Reese-Ellsworth classification); 30-40% for group IV and 10-15% for patients with advanced group V disease.

Of patients previously treated with EBRT, 60% require further therapy with cryotherapy or photocoagulation.

Of patients requiring treatment with EBRT, 20% eventually require enucleation.

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Patient Education

Genetic counseling for retinoblastoma

In 1979, Vogel published his review on the genetics of retinoblastoma in the Journal of Human Genetics. He reviewed the likelihood for the recurrence of retinoblastoma in close relatives of a patient with the disease, based on clinical criteria, as shown below. It is the physician's responsibility to inform the patient's family that retinoblastoma can be hereditary. The methods for screening and estimation of risks are highly improved with use of molecular genetics techniques, although this sometimes can prove to be very expensive.

Genetic counseling for retinoblastoma. (This table Genetic counseling for retinoblastoma. (This table is modified from Vogel F. Genetics of retinoblastoma. Hum Genet 1979; 52:1.)

Normal individuals have 2 copies of the retinoblastoma gene, 1 coming from each parent. However, in patients with retinoblastoma, one copy of the gene is inactivated by an initial mutation.

When the initial mutation arises from a somatic cell (retinal), the patient has the nonhereditary type of retinoblastoma and the relatives have a low risk for the disease. These individuals have 1 abnormal gene in all their cells, and the mutation in the other gene (in the retinal cell) allows the expression of the tumor.

When the initial mutation arises from the germline, the patient has the hereditary type of retinoblastoma and the relatives of the patient have a significant risk for retinoblastoma. In these individuals, both mutations occur only in the retinal cell that has become malignant.

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