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Orbital tumors
can be benign or malignant and arise primarily within the orbit
or secondarily from an adjacent source, such as the eyelid, paranasal
sinus, or intracranial compartment.
Causes differ by age. The more common benign pediatric tumors include dermoid tumors and vascular lesions such as capillary hemangioma and lymphangioma. In adults, cavernous hemangiomas predominate.
Some orbital tumors usually cause proptosis and displacement of the globe in a direction opposite the tumor. Pain, diplopia, and vision loss may also be present. Diagnosis, in most cases, is based on the history, examination, and neuroimaging (CT, MRI, or both).
Treatment
Treatment varies by tumor type. Treatment of dermoid tumors is excision. Capillary hemangiomas tend to spontaneously involute and therefore do not need any treatment; however, especially when located on the upper eyelid, they may affect vision and require treatment with interlesional injection of corticosteroids or surgical debulking.
Children:
The common pediatric malignant tumors include rhabdomyosarcoma and metastatic lesions related to leukemia or neuroblastoma. If rhabdomyosarcoma is resectable, then surgery is done, followed by chemotherapy and orbital radiation therapy. Leukemic disease is usually managed by orbital radiation therapy, chemotherapy, or both.
Adults:
The most common benign tumors are meningiomas, mucoceles, and cavernous hemangiomas. Sphenoid wing meningiomas are treated with debulking via craniotomy when symptomatic, sometimes followed by a course of radiation therapy. Because meningioma cells infiltrate bone of the skull base, complete resection usually is not possible. Mucoceles are treated by draining the offending lesion into the nose, because they most commonly arise from the ethmoid or frontal sinus. Cavernous hemangiomas are excised.
Common malignant tumors include lymphoma, squamous cell carcinoma, and metastatic disease. Lymphomas involving the orbit are typically B-cell and characteristically low grade. Lymphomas can be bilateral and simultaneous and can be part of a systemic process or exist in the orbit in isolation. Radiation therapy effectively treats orbital lymphomas with few adverse effects, although the addition of monoclonal antibodies against a surface receptor (CD20) on the lymphocyte is also effective. Most squamous cell carcinomas arise from the adjacent paranasal sinuses. Surgery, radiation therapy, or both form the backbone of therapy. Metastatic disease is usually treated with radiation therapy. Metastatic disease involving the orbit is usually an unfavorable prognostic sign, with carcinoid tumors being a notable exception.
Last full review/revision September 2008 by James Garrity, MD
Content last modified September 2008
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