Laterality

Bilateral Tumors

Patients have one or more tumors in both eyes. Tumor development can be synchronous or metachronous. It is presumed that these patients have the hereditary form of the disease even in the absence of a positive family history. These patients almost always present prior to age 2 years and most present in the first year of life. The severity of tumors may be variable in the two eyes, and this becomes important in weighing potential treatment options.

Unilateral Tumors

Patients have one or more tumors in one eye. Median age of presentation is 23 months, with few presenting under 1 year of age. Tumor development within the affected eye can be synchronous or metachronous. For those with unifocal unilateral disease, it is presumed that these patients have the nonhereditary form of the disease. Patients with multifocal unilateral disease or those who present at a younger age are more likely to have the hereditary form of the disease.

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