Written by Paul T. Finger, MD
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 common 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 rule out other diseases that cause orbital inflammation.
Orbital pseudotumor is typically characterized by the rapid development of pain, proptosis, and swelling around the eye and orbit. 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. These cases typically respond rapidly to steroid therapy (which helps confirm the diagnosis). Atypical cases of orbital pseudotumor usually undergo biopsy which helps establish the diagnosis.
It is important 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). A biopsy specimen 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 can return. Eye cancer specialists usually 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:
- Thyroid Eye Disease
- Infectious Orbital Cellulitis
- Orbital Myositis
- Orbital Vasculitis
- Sjogren's Disease
- Wegener's Granulomatosis
- Malignant Ocular Tumors