Sclerosing orbital pseudotumor is uncommon. Due to unknown reasons, these tumor behave differently than other types of pseudotumor of the orbit. They grow more slowly, cause less pain, and are characterized by scarring (hardening of the tumor tissue).
Sclerosing orbital pseudotumor is not cancer. But, by local growth it can cause bulging of the eye (proptosis), double vision (diplopia) and loss of vision. Sclerosing orbital pseudotumor can (rarely) extend into the sinuses, brain, and other orbit.
Sclerosing orbital pseudotumor is usually diagnosed by biospy (orbitotomy).
Once the diagnosis is confirmed by pathology, systemic testing to rule out specific infectious and inflammatory causes should be performed. For example, an ANCA blood test and a chest x-ray should be performed to rule out Wegener’s Granulomatosis. Many of these patients have a past medical history of sinusitis, sinus surgery, or inhalation drug abuse. Therefore, concurrent treatable sinus disease should be addressed.
Sclerosing orbital pseudotumors are less responsive to steroid therapy. Most cases are treated with combinations of surgery, steroid therapy, radiation and chemotherapy depending upon the clinical picture and the patient’s response to treatment.
Inflammations can affect the tissues around the eye (orbit and adnexa). Certain orbital inflammations can look like tumors and are therefore called orbital pseudotumor. Orbital pseudotumor can affect one or both eyes of relatively young patients (less than 50 years old). They are not cancer.
Orbital pseudotumor can be quite painful. In fact, pain is one of the most prominent characteristics of this disease. In addition to pain, an inflammatory mass (tumor) can make the patient’s eye protrude (proptosis) and restrict the movement of the eye. A biopsy (called an orbitotomy) is commonly performed to confirm the diagnosis of orbital pseudotumor and to obtain tissue for pathology examination.
Orbital pseudotumor is typically characterized by the rapid development of pain, proptosis, and swelling around the eye and orbit in adults. Ultrasound and computed tomographic (CT) scanning typically shows a diffuse infiltration of the orbit, an inflammation of the eye wall (sclera), and/or T-sign (with the optic nerve). Orbital pseudotumor related orbital masses typically have poorly defined margins. Systemic testing (blood and spinal fluid) may show signs of inflammation (e.g. increased sedimentation-rate) or atypical cells.
Patients with classic findings of orbital pseudotumor may be treated without a biopsy. A rapid complete response to steroid therapy helps confirm the diagnosis. Atypical cases of orbital pseudotumor usually undergo a diagnostic biopsy.
Specimens can be sent to test for infectious causes of orbital inflammation and certain systemic diseases. Typically eye cancer specialists will obtain blood, skin and radiographic (e.g. x-ray, MRI) tests for a variety of diseases such as sarcoidosis, tuberculosis, and Wegener’s Granulomatosis (see table below). An orbital biopsy can be particularly helpful in diagnosing many of these disorders.
Orbital pseudotumor will respond rapidly to high-dose steroid therapy. Unfortunately, when the steroids are stopped, the inflammation often returns. Eye cancer specialists must reduce the steroid medication very slowly in order to prevent recurrence (return) of the disease.
In certain cases, chemotherapy (e.g. methotrexate, cyclosporine) and low-dose radiation (e.g. 1500-2500 cGy EBRT) may be needed to control the inflammation related to orbital pseudotumor. Most patients do well with steroid therapy but they are always at risk for recurrent orbital pseudotumor.
Other Common Causes of Orbital Swelling and Inflammation:
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