Orbital Tumors and Disorders

Orbital Tumors Information

What are Orbital Tumors and Disorders?

The orbit contains the bones of the eye socket, the eyeball, the eye socket muscles, the optic nerve, and the surrounding fat. Any of these structures can become inflamed or develop a tumor or lesion, resulting in a wide range of symptoms, such as a bulging eye (known as proptosis), double vision, or vision loss. The underlying cause of the condition and/or the location and size of the tumor will determine the best treatment option.

State-of-the-art Care from Leading Experts

At the Center for Thyroid Eye Disease and Orbital Surgery, our team of renowned surgeons, pathologists, and radiologists has the expertise to make accurate diagnoses and provide comprehensive care for orbital tumors and disorders. They are also well-equipped to provide highly specialized care for rare orbital tumors, including vascular and lymphatic malformations.

Additionally, our surgeons have developed novel, minimally invasive surgical techniques to remove complex tumors from behind the eye. Instead of making a large incision near the eye and removing part of the bone, doctors are often able to perform surgery through the nose. This results in a shorter hospital stay (many patients are able to go home in less than 24 hours), less pain, and improved function and appearance.

Read below to learn more about the types of orbital tumors and conditions we treat.

Inflammatory Orbital Disorders

Thyroid eye disease (thyroid-associated orbitopathy): This autoimmune inflammatory process in the orbit most commonly occurs with Graves’ disease, but it can also occur when thyroid hormone levels are low or even normal. Symptoms include dry eyes, bulging of the eyes (caused by the eyes displacing forward or from the eyelids pulling back), double vision, and vision loss. The treatment can be medical or surgical. Learn more about minimally invasive treatments at the center.

Orbital cellulitis: Orbital cellulitis is an infection of tissues around the eye and within the eye socket. It occurs when an infectious organism from elsewhere in the body (such as the nasal sinuses, skin, or bloodstream) spreads to the eye socket. Symptoms may include bulging eyes, difficulty moving the eye, and vision loss.

Idiopathic orbital inflammatory syndrome (orbital pseudotumor): This painful, benign (noncancerous) inflammation of the tissues around the eye may include the tear gland, the eye wall (sclera), or the eye muscles. Blood tests are typically used to try to determine the inflammatory disease causing the problem. A biopsy might also be performed. Treatment often includes steroids or other anti-inflammatory medications.

Orbital sarcoidosis: Sarcoidosis is a systemic disease that can affect any body system, most commonly the lungs, skin, or lymph nodes. In rare cases, symptoms may occur in the orbit. Most commonly the tear gland becomes inflamed, causing drooping or swelling of the eyelid, although other tissue in the orbit can also be involved. Additional symptoms may include bulging eye or double vision. A biopsy can be performed, and lab tests and a chest scan can help confirm the diagnosis.

Orbital Wegener’s granulomatosis (granulomatosis with polyangiitis): This autoimmune inflammatory disorder can involve any organ system, but it most commonly affects the lungs, kidneys, and the head and neck, including the eye and orbit. If this disease occurs in the orbit, the eye can be pushed forward, or double vision or vision loss may occur. Blood tests and CT scans may be used to confirm the diagnosis. The condition is treated with anti-inflammatory medications.

Cystic Orbital Lesions

Orbital dermoid cyst: Dermoid cysts are common, benign, slow-growing cysts that usually occur on the boney rim of the orbit. They may cause a firm nodule, drooping of the eyelid, or even double vision. They are present at birth and are most commonly seen in children. Sometimes they present after rupturing and causing inflammation of the surrounding skin. These cysts can be surgically removed.

Orbital teratoma (teratomatous cyst): Orbital teratomas, which are quite rare, are often present at birth and are usually benign. They contain many different types of tissues from different body systems. Orbital teratomas may be very large and disfiguring. A CT scan or MRI is done to confirm the diagnosis, and the cyst is removed surgically.

Orbital mucocele: A mucocele is a fluid-formed cyst that develops from the lining of the nasal sinuses. These cysts can grow into the orbit, causing the eye to be displaced. Additional symptoms include double vision or loss of vision. A CT scan can help to diagnose the condition. Patients typically undergo surgery involving the sinuses and the eye socket.

Vascular Orbital Lesions

Orbital capillary hemangioma: This common, benign lesion in the orbit affects children, often in the eyelids, but it can extend into the orbit. Depending on the depth of the lesion, it may appear as a raised red lesion or a blue lesion on the eyelid. These lesions may cause the eyelid to droop, making it difficult to use the eye. An ultrasound, CT scan, or MRI may be done to determine the depth of the lesion. Treatment may be medical—with a laser orembolization—or surgical. A pediatric ophthalmologist may be needed in some cases to monitor visual development.

Orbital cavernous hemangioma: This benign, slow-growing tumor is composed of blood vessels. Often seen in middle-aged patients, it may be discovered incidentally when a CT scan or MRI is done for other reasons. Treatment, which involves surgical excision, is usually only necessary if vision is compromised.

Orbital hemangiopericytoma: This rare connective tissue tumor can be located behind the eye, in the eyelid skin, or in the tear sac. Generally slow-growing, but sometimes malignant, this type of tumor may cause the eye to bulge or lead to double vision, orbital pressure or pain, or a mass in the corner of the eye near the nose. Tearing or bloody tears may occur if the tear sac is involved. A CT scan and MRI are done to visualize the lesion. Surgery may be indicated.

Orbital lymphangioma (lymphatic malformation): These benign lesions are a collection of fluid-filled cysts that are present at birth, but may not be evident until later in life. They can occur on the surface of the eye, the eyelids, and in the orbit, often leading to problems such as forward displacement of the eye, disfigurement of the eyelids, and vision loss. These lesions can sometimes progress rapidly in a few days if there is bleeding within the lesion. Treatment may include medications, draining the lesion, injecting agents into the cysts, or surgery.

Orbital varix: This benign lesion is present at birth and is composed of a mass of blood vessels. This may cause forward displacement of the eye, disfigurement of the eyelids, and vision loss. Treatment is usually only necessary if there is vision loss, double vision, or disfigurement, and it involves draining the lesion, injecting agents into the cysts, or surgery.

Orbital angiosarcoma: This rare, malignant (cancerous) connective tissue tumor can be located in the orbit. It may cause forward displacement of the eye or drooping of the eyelid. A CT scan or MRI is performed, and treatment involves surgery, which is often combined with radiation or chemotherapy.

Orbital arteriovenous malformation or fistula: This benign lesion is caused by an abnormal connection between veins and arteries. This may cause forward displacement of the eye, disfigurement of the eyelids, and vision loss. Symptoms may fluctuate over time. If there is vision loss, double vision, or disfigurement, treatment may include draining the lesion, injecting agents into the cysts, or surgery.

Orbital Fibrous Connective Tissue Tumors

Orbital nodular fasciitis and fibroma: This benign overgrowth of connective tissue can cause a mass in the eyelid, on the boney wall of the eye, or in the orbit. A CT scan or MRI is performed, but the mass is usually removed surgically to confirm the diagnosis.

Orbital solitary fibrous tumor (orbital fibrous histiocytoma): This rare connective tissue tumor can be located in the orbit. Generally slow-growing, but sometimes malignant, this type of tumor may cause forward displacement of the eye, double vision, or drooping or swelling of the eyelid. A CT scan and MRI are done to visualize the lesion, and if surgery is indicated, the diagnosis can be confirmed by examining the removed tissue.

Orbital fibrosarcoma: This is a rare, malignant connective tissue tumor. If it is located in the orbit, symptoms may include forward displacement of the eye, double vision, or drooping or swelling of the eyelid. A CT scan and MRI are done to visualize the lesion. Surgery to remove the tumor may involve removing surrounding bone. Radiation and chemotherapy may be necessary. The diagnosis is made by examining the removed tissue.

Neural and Meningeal Lesions

Orbital schwannoma: This slow-growing, benign tumor develops within the outer covering of nerves. It may cause forward displacement of the eye, double vision, vision loss, or drooping or swelling of the eyelid. These tumors can be associated with neurofibromatosis (nervous system tumors). Surgery to remove the tumor can be done for symptomatic tumors, and the diagnosis is made by examining the removed tissue.

Orbital neurofibroma: This tumor is composed of the outer covering of nerves and connective tissue. These tumors can be associated with neurofibromatosis type 1 (a disorder that causes tumors to grow in the nervous system). Orbital symptoms include forward displacement of the eye, double vision, or drooping or swelling of the eyelid. A CT scan and MRI are done to visualize the lesion, and surgery may be indicated.

Orbital granular cell tumor: This rare tumor is thought to be composed of the outer covering of nerves and connective tissue. If the tumor is located in the orbit, it may cause restricted eye movement and double vision. In some cases, it may be malignant. Surgical removal can be undertaken, but it is difficult to excise the tumor completely and it may recur. The diagnosis is made by examining the removed tissue. Radiation and chemotherapy may be used to prevent recurrence.

Orbital malignant peripheral nerve sheath tumor: This rare, malignant tumor is composed of the outer covering of nerves and can be associated with neurofibromatosis. When this tumor develops in the orbit, it may cause forward displacement of the eye, double vision, vision loss, and drooping or swelling of the eyelid. The tumor is removed surgically, and the diagnosis is made by examining the removed tissue.

Optic nerve juvenile pilocytic astrocytoma (optic nerve glioma): Optic nerve glioma is a slow-growing tumor that typically affects children. One-third of patients have associated neurofibromatosis type 1. Malignant gliomas (glioblastoma) are rare and almost always occur in adults. Symptoms may include forward displacement of the eye, double vision, or painless vision loss. Treatment options include observation, surgical excision, radiation, or chemotherapy.

Optic nerve sheath meningioma: These rare tumors originate from the lining of the optic nerve. They are usually benign but may be malignant in rare cases. The tumor may cause slow, progressive vision loss due to compression of the optic nerve and its blood supply. This tumor may be associated with exposure to radiation or neurofibromatosis type 2. Treatment options are observation, radiation, or surgical removal of the tumor.

Sphenoid wing meningioma: These slow-growing tumors originate from the lining of the brain. They can spread through the sphenoid bone into the orbit and may cause forward displacement of the eye, double vision, or vision loss. They may be associated with neurofibromatosis type 2. Treatment options may include observation, radiation, or surgical removal of the tumor.

Primary orbital neuroblastoma: This rare tumor is derived from cells, called neuroblasts, and is most commonly seen in children. Symptoms include forward displacement of the eye, double vision, or drooping or swelling of the eyelid. The tumor is removed surgically, and the diagnosis is made by examining the removed tissue.

Myogenic Tumors of the Orbit

Orbital rhabdomyosarcoma: This rare, malignant childhood cancer occurs behind the eye in the orbit. It may be associated with past radiation treatment. Symptoms may include forward or downward displacement of the eye, bruising or swelling of the eyelids, vision loss, or difficulty moving the eye. The tumor is surgically removed and biopsied, and additional treatment may include chemotherapy and possibly radiation.

Orbital leiomyoma: This rare, benign tumor originates from muscle. It may cause forward displacement of the eye or double vision. The appearance of this tumor on a CT scan or MRI looks like many other orbital tumors. Management may include observation, biopsy, or excision.

Orbital leiomyosarcoma: This rare, benign tumor originates from muscle. It may cause forward displacement of the eye or double vision. This tumor interweaves with other orbital tissues. A biopsy is needed to make a diagnosis. Surgical treatment often involves removing surrounding tissues.

Orbital Osseous, Fibro-osseus, and Cartilaginous Tumors

Orbital osteoma: This benign tumor originates from bone. It may cause forward displacement of the eye, visible deformity, vision loss, or difficulty moving the eye. The tumor is surgically removed (excisional biopsy), and the diagnosis is made by examining the removed tissue.

Orbital osteosarcoma: This rare malignant, tumor originates from bone. It may cause forward displacement of the eye, visible deformity, vision loss, or difficulty moving the eye. A biopsy is needed to make a diagnosis. Treatment is a combination of surgery, radiation, and chemotherapy.

Orbital fibrous dysplasia: This is a disorder in which the orbital bone is replaced by an overgrowth of abnormal bone. Symptoms include pain, forward displacement of the eye, double vision, and disfigurement, with loss of vision occurring late in the course of the disease. There may also be problems with the adjacent sinuses or nerves, which can result in hearing loss. Management may be observation, medical treatment, or surgery.

Orbital ossifying fibroma: This benign tumor originates from bone. It may cause displacement of the eye, double vision, drooping of the eyelid, or inflammation in the orbit that mimics an infection. The tumor is surgically removed, and the diagnosis is made by examining the removed tissue.

Orbital chondroma: This rare benign tumor is composed of cartilage. It may cause forward displacement of the eye, visible deformity, or drooping of the eyelid. The tumor is surgically removed, and the diagnosis is made by examining the removed tissue.

Orbital chondrosarcoma: This rare malignant, tumor originates from cartilage. It may cause forward displacement of the eye, pain, visible deformity, loss of vision, or difficulty moving the eye. Treatment is a combination of surgery, radiation, and chemotherapy, and the diagnosis is made by examining the removed tissue.

Orbital Lipomatous and Myxomatous Tumors

Orbital fat prolapse: The orbital fat behind the eye may prolapse forward, creating visible yellow elevations that are seen on the upper outer surface of the eye and under the upper eyelid. This may cause a visible deformity of the eyelid or result in ocular irritation. The prolapse can occur after surgery, trauma, or spontaneously due to age-related changes. Treatment is observation or surgical removal of the fat.

Orbital/conjunctival dermolipoma: This lesion is present at birth and is composed of skin cells and fat. It is often located on the upper outer surface of the eye, but unlike an orbital fat prolapse, dermolipoma occurs in younger patients, has a pinkish appearance, and does not move as freely as orbital fat. Removal can be complex, and in some cases, may cause dry eye or double vision. These lesions do not enlarge and are usually observed, unless the patient has symptoms.

Orbital lipoma and angiolipoma: This benign tumor is composed of fat and sometimes connective tissue and blood vessels. It may cause forward displacement of the eye, visible deformity, or difficulty moving the eye. The tumor may be surgically removed, and the diagnosis is made by examining the removed tissue.

Orbital liposarcoma: This malignant tumor originates from fat. It may cause forward displacement of the eye, visible deformity, or difficulty moving the eye. The tumor is surgically removed, and the diagnosis is made by examining the removed tissue.

Orbital Histiocytic Tumors and Pseudotumors

Orbital juvenile xanthogranuloma: This tumor may occur as a solitary mass in the orbit in infants. Symptoms may include forward displacement of the eye, visible deformity, or difficulty moving the eye. The tumor is surgically removed, and the diagnosis is made by examining the removed tissue.

Orbital Langerhans cell histiocytosis (eosinophilic granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease): This is a group of diseases, ranging from benign to malignant, that originate from an immune system cell called a Langerhan cell. It usually occurs in children, causing redness around the eye and pain, and it may involve other body systems, including the skin, bone, and lungs. A biopsy is done to make the diagnosis and treatment may involve observation or chemotherapy.

Orbital Erdheim-Chester disease (non-Langerhans cell histiocytosis): This disease is seen in older adults and presents in the orbit as yellowish plaques in the eyelid skin and forward displacement of the eyes. This disease is due to the overgrowth of an immune system cell called a histiocyte. Many parts of the body are affected, often with bone, kidney, skin, brain, and lung involvement. Bone pain is common. A biopsy is performed to make the diagnosis and treatment is a combination of surgery, chemotherapy, immune-suppressing medications, and radiation.

Orbital Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy): This disease is due to overgrowth of an immune system cell called a histiocyte. It may result in forward displacement of one or both eyes, vision loss, eyelid swelling, a rubbery mass, large lymph nodes, or fever. Treatment may involve observation, surgery, chemotherapy, immune-suppressing medications, and radiation.

Orbital necrobiotic xanthogranuloma: This disease causes yellowish plaques on the eyelid skin, but unlike cholesterol plaques, they are firmer and can even ulcerate. There is often a high level of abnormal protein in the blood, and it is associated with other diseases, including multiple myeloma. Treatment may involve chemotherapy, immune-suppressing medications, and radiation.

Orbital Primary Melanocytic Tumors

Orbital melanoma: Rarely occurring in the orbit, this malignant tumor originates from a pigmented cell called a melanocyte. This tumor may occur in eyes that have an unusual amount of pigment, a condition called ocular melanocytosis. It may cause displacement of the eye, vision loss, or difficulty moving the eye. The tumor is surgically removed, and the diagnosis is made by examining the removed tissue. Afterward chemotherapy or other medications may be required.

Lacrimal Gland Lesions

Dacryops (lacrimal gland ductal epithelial cyst): This benign cyst occurs in one of the drains from the major tear gland. It develops as a non-tender mass at the outer corner of one or both eyes, which may cause a visible mass in the upper eyelid. These masses may wax and wane over time. Treatment may include observation or surgery. However, surgery is usually avoided because there is a risk of causing severe dry eye.

Lacrimal gland pleomorphic adenoma (benign mixed tumor): This benign tumor occurs in the tear gland and is composed of a variety of tissue types. This may result in a painless mass in the upper outer eyelid, drooping eyelid, double vision, or forward displacement of the eye. In some cases, the tumor may recur after removal or become malignant. Therefore, complete surgical removal is important, and the diagnosis is made by examining the removed tissue.

Lacrimal gland pleomorphic adenocarcinoma: This malignant tumor occurs in the tear gland and is composed of a variety of tissue types. This tumor may occur from a benign pleomorphic adenoma or may occur as a new tumor. The tumor may present as a painless mass in the upper outer eyelid, causing a drooping eyelid, double vision, or forward displacement of the eye. The tumor is surgically removed, and the diagnosis is made by examining the tissue removed. Adjunctive treatment, such as radiation, may be required.

Lacrimal gland adenoid cystic carcinoma: This malignant tumor occurs in the tear gland. The tumor may present as a mass in the upper outer eyelid, causing a drooping eyelid, double vision, forward or downward displacement of the eye, and pain or loss of feeling if the bone or nerves are involved. The tumor and surrounding tissues are surgically removed, and the diagnosis is made by examining the removed tissue. Afterward, chemotherapy or radiation may be needed.