Orbital tumors represent a topic of vital importance for the clinician in ophthalmology, because its timely diagnosis affects the patient´s prognosis. These tumors can appear at any age, but fortunately they are very rare so they have a really low incidence in population.
An orbital tumor refers to any tumor located in the orbit; this is the bony cavity that contains the eyeball and its associated structures (muscles, vessels, nerves).
What is the Main Cause of Orbital Tumor?
Researchers suggest that the most frequent causes of orbital tumors are neoplastic, which were explained here in detail. In a second place we have the inflammatory causes (finding that inflammatory pseudotumor as the most common) while vascular causes (Lymphangioma standing out above the other types) occupy the last place in frequency.
The content of the orbit is organized in such a way that it does not allow lodging occupational injuries, for which they produce diverse signs and symptoms. Proptosis is the most important clinical manifestation of orbital disease. Other representative clinical manifestations are alterations in eye movements, which can provoke plejias and diplopia. Other symptoms are variations in visual acuity, conjunctival injection, chemosis, pain and pupillary changes, among others.
After the clinical evaluation, is fundamental performing image studies to evaluate the injury and its extension in order to plan the biopsy. It is relevant to take an adequate amount of tissue for anatomopathological and immunohistochemical analysis.
This will give the diagnosis of certainty. Computed tomography of orbit shows an irregular or circumscribed lesion that molds structures that surround it without eroding the bone.
Experts recommend that to manage orbital tumors in a proper way we must take into account the nature of the lesion. These tumors have had an adequate response to radiation therapy or chemotherapy, although in most cases surgery is required.
These tumors can be organized according to their provenance in primary lesions which originate from the orbit itself, secondary lesions, which expand to the orbit from nearby structures and finally, in metastatic tumors (when metastasis occurs, cancerous cells separate from the primary tumor, travel through the blood or lymphatic system and constitute a new tumor in other body tissues).
A great variety of tumors have its origin in the orbit. Vascular, lymphoid, nervous and mesenchymal structures are normally found inside of it, being able to originate primary tumors. In a scientific study the five primary tumors found were hemangioma, non-Hodgkin’s lymphoma, inflammatory tumors, meningioma and optic nerve glioma.
The anatomical proximity of the orbit with other relevant structures such as the paranasal sinuses, skull, conjunctiva, sac and lacrimal gland, eyelids and the eyeball itself make the secondary invasion a common cause of orbital tumors. The most common secondary tumors are mucoceles, squamous cell carcinoma, meningioma, vascular malformations, and basal cell carcinoma, among others.
Metastatic disease, particularly adenocarcinoma of the lung and breast, can cause orbital lesions. In addition to metastatic lesions, some inflammatory diseases and systemic vasculitis can produce masses within the orbit.
It is important to mention that a considerable number of orbital tumors affect the pediatric group, which are usually benign. Depending on the study analyzed, between 10% and 30% of orbital tumors in childhood are malignant. However, among the most common benign lesions are dermoid cysts, vascular tumors (capillary hemangioma and lymphangioma), glioma of the optic nerve and inflammatory tumors.
The most frequent malignant tumors are rhabdomyosarcoma, metastatic neuroblastoma (studies report that they particularly are the most common metastatic tumor that expands to the orbit), infiltration by leukemia, Ewing’s sarcoma and extraocular extensions of retinoblastoma, all of which are causes of malignant tumors in this age group.
On the other hand, in adults, the most common primary tumors to metastasize to bone are: breast, lung, prostate, melanoma, gastrointestinal tract, and kidney.
- American Academy of Ophthalmology. Orbital Tumors. https://www.aao.org/eye-health/diseases/orbital-tumors
- National Center for Biotechnology Information. Orbital Tumors: Diagnosis and Management. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4641017/