Some types of 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. The diagnosis of orbital tumors is suspected based on the history, examination, and neuroimaging (CT, MRI, or both), but confirmation often ultimately requires a biopsy. Causes and treatment vary by age group.
Benign pediatric tumors are most commonly dermoid tumors and vascular lesions such as infantile hemangioma Infantile Hemangiomas Infantile hemangiomas are raised, red or purplish, hyperplastic vascular lesions appearing in the first year of life. Most spontaneously involute; those obstructing vision, the airway, or other... read more and lymphatic malformations Lymphatic Malformations Lymphatic vascular malformations are elevated lesions composed of dilated lymphatic vessels. This photo shows a cystic hygroma, or lymphatic malformation, on the neck of a 2-year-old girl. This... read more . Treatment of dermoid tumors is excision. Infantile hemangiomas tend to spontaneously involute and therefore usually do not need any treatment; however, especially when located on the upper eyelid, they may affect vision and require treatment with systemic beta-blockers. Small lymphatic malformations that do not cause symptoms may be followed clinically. For larger lymphatic malformations or those that are causing symptoms, options include surgical debulking, intralesional sclerotherapy, and sirolimus (1 General reference 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. Orbital... read more ) .
Malignant pediatric tumors are most commonly rhabdomyosarcoma Rhabdomyosarcoma Rhabdomyosarcoma is a cancer arising from embryonal mesenchymal cells that have potential to differentiate into skeletal muscle cells. It can arise from almost any type of muscle tissue in any... read more and metastatic lesions related to leukemia Overview of Leukemia Leukemia is a malignant condition involving the excess production of immature or abnormal leukocytes, which eventually suppresses the production of normal blood cells and results in symptoms... read more or neuroblastoma Neuroblastoma Neuroblastoma is a cancer arising in the adrenal gland or less often from the extra-adrenal sympathetic chain, including in the retroperitoneum, chest, and neck. Diagnosis is confirmed by biopsy... read more . If rhabdomyosarcoma is resectable, surgery is done, followed by chemotherapy and orbital radiation therapy. Leukemic disease is usually managed by orbital radiation therapy, chemotherapy, or both.
Benign adult tumors are most commonly meningiomas Meningiomas Meningiomas are tumors of the meninges that can compress adjacent brain tissue. Symptoms depend on the tumor’s location. Diagnosis is by MRI with contrast agent. Treatment may include excision... read more , mucoceles, and cavernous venous malformations (previously known as cavernous hemangiomas Infantile Hemangiomas Infantile hemangiomas are raised, red or purplish, hyperplastic vascular lesions appearing in the first year of life. Most spontaneously involute; those obstructing vision, the airway, or other... read more ). Pleomorphic adenomas of the lacrimal gland are less common. When symptomatic, sphenoid wing meningiomas are treated with debulking via craniotomy, 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 them into the nose because they most commonly arise from the ethmoid or frontal sinus. Cavernous venous malformations (the most common benign orbital tumor) and lacrimal gland pleomorphic adenomas are excised.
Malignant adult tumors are most commonly lymphoma Overview of Lymphoma Lymphomas are a heterogeneous group of tumors arising in the reticuloendothelial and lymphatic systems. The major types are Hodgkin lymphoma Non-Hodgkin lymphoma See table Comparison of Hodgkin... read more , squamous cell carcinoma Squamous Cell Carcinoma Squamous cell carcinoma is a malignant tumor of epidermal keratinocytes that invades the dermis; this cancer usually occurs in sun-exposed areas. Local destruction may be extensive, and metastases... read more , and metastatic disease. Less commonly, the tumor is an adenoid cystic carcinoma of the lacrimal gland, which is an aggressive tumor.
Lymphomas involving the orbit are the most frequent malignant orbital tumor and are typically B-cell and characteristically low grade (usually a MALT lymphoma [mucosa-associated lymphoid tissue], also known as extranodal marginal zone B-cell lymphoma). Lymphomas can be bilateral and simultaneous and can be part of a systemic process or exist in the orbit in isolation. Many orbital lymphomas have minimal symptoms and findings despite impressive radiologic findings. Radiation therapy effectively treats orbital lymphomas with few adverse effects, but treatment with monoclonal antibodies (eg, rituximab) against a surface receptor (eg, CD20) on the lymphocyte is also effective and should be considered in addition to or instead of radiation therapy, particularly if lymphoma is systemic.
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; carcinoid tumors are a notable exception.
Lacrimal gland adenoid cystic carcinoma is treated with surgery and then usually with radiation therapy (sometimes proton beam therapy) or by a protocol using intra-arterial chemotherapy with radiation therapy and surgery.
1. Shoji MK, Shishido S, Freitag SK: The use of sirolimus for treatment of orbital lymphatic malformations: A systematic review. Ophthalmic Plast Reconstr Surg 36:215-221, 2020. doi: 10.1097/IOP.0000000000001518
Drugs Mentioned In This Article
|RIABNI, Rituxan, RUXIENCE, truxima