Metastatic Tumors in Children
In children, distant tumors metastasize to the orbit more frequently than to the globe (in contrast to adults, who more frequently have metastases to the choroid). Tumors that can metastasize to the orbit in children include Burkitt lymphoma, leukemia, neuroblastoma, Ewing sarcoma, and Wilms tumor (nephroblastoma).
In advanced stages, leukemia may produce unilateral or bilateral proptosis. Acute lymphoblastic leukemia is the type of leukemia most likely to metastasize to the orbit. A primary leukemic orbital mass, called granulocytic sarcoma or chloroma, is a rare variant of myelogenous leukemia. Least common are metastases to the subarachnoid space of the optic nerve. These cases present with sudden vision loss and swelling of the optic nerve. They constitute an emergency and are treated with orbital radiotherapy.
Typically, orbital lesions present before blood or bone marrow signs of leukemia, which almost invariably follow within several months. Special stains for cytoplasmic esterase in the cells (Leder stain) indicate that they are granulocytic precursor cells. Chances for survival are improved if chemotherapy is instituted before the discovery of leukemic involvement in bone marrow or peripheral blood.
Metastatic orbital neuroblastoma occurs in 10%–20% of cases and typically produces an abrupt ecchymotic proptosis that may be bilateral. A deposition of blood in the eyelids may lead to the mistaken impression of injury (Fig 5-25). Horner syndrome may also be evident in some cases. Commonly, bone destruction is apparent on CT, particularly in the lateral orbital wall or sphenoid marrow. Metastases typically occur late in the course of the disease, when the primary tumor can be detected readily in the abdomen, mediastinum, or neck. Treatment consists primarily of chemotherapy; radiotherapy is reserved for cases of impending vision loss due to compressive optic neuropathy. The survival rate of neuroblastoma is related to the patient’s age at diagnosis. Patients diagnosed before 1 year of age have a significantly better prognosis (a 5-year survival of 85%) than children diagnosed after 1 year of age (5-year survival rate of 36%). Unfortunately, the average age of presentation of patients with orbital metastases is over 2 years of age. Congenital neuroblastoma of the cervical ganglia may produce an ipsilateral Horner syndrome with heterochromia.
Figure 5-25 Metastatic neuroblastoma. A, Child with metastatic left orbital neuroblastoma. B, T2-weighted MRI shows a large infiltrating lesion of the left sphenoid wing extending into the orbital soft tissues and the temporal fossa.
(Courtesy of Bobby S. Korn, MD, PhD.)
Aggarwal E, Mulay K, Honavar SG. Orbital extra-medullary granulocytic sarcoma: clinicopathologic correlation with immunohistochemical features. Surv Ophthalmol. 2014;59(2): 232–235.
Ahmed S, Goel S, Khandwala M, Agrawal A, Chang B, Simmons IG. Neuroblastoma with orbital metastasis: ophthalmic presentation and role of ophthalmologists. Eye (Lond). 2006;20(4): 466–470.
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.