Neuroblastoma is one of the most common childhood cancers and is the most frequent source of childhood orbital metastasis (89% of cases). It usually originates in either the adrenal gland or the sympathetic ganglion chain in the retroperitoneum or mediastinum. Approximately 20% of all patients with neuroblastoma show clinical evidence of orbital involvement, which is sometimes the initial manifestation of the tumor.
The mean age at diagnosis of patients with metastatic orbital neuroblastoma is approximately 2 years; 90% are diagnosed by 5 years of age. Unilateral or bilateral proptosis and eyelid ecchymosis are the classic presentations (Fig 28-29). Systemic signs and symptoms may include abdominal fullness and pain, venous obstruction and edema, hypertension caused by renal vascular compromise, and bone pain. Urinalysis for catecholamines is positive in 90%–95% of cases.
Figure 28-28 Leukemic infiltration of the optic nerve.
Figure 28-29 Bilateral orbital metastasis from neuroblastoma in a 2-year-old girl, presenting with periorbital ecchymosis.
Figure 28-30 Right Horner syndrome, the presenting sign of localized intrathoracic neuroblastoma in a 6-month-old boy.
Opsoclonus, characterized by rapid, multidirectional saccadic eye movements, is a paraneoplastic syndrome that is associated with neuroblastoma and is not related to orbital involvement. It is associated with a good prognosis for survival, but neurologic deficits may persist. Horner syndrome can occur as a result of a primary cervical or apical thoracic neuroblastoma that involves the sympathetic chain (Fig 28-30).
Treatment modalities include surgery, chemotherapy, and radiation therapy. Neuroblastoma that presents in a child younger than 1 year has a more favorable prognosis than that in older children. Approximately 10% of neuroblastomas undergo spontaneous regression.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.