Linfomas y Enfermedad Inflamatoria de la Orbita

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1 Linfomas y Enfermedad Inflamatoria de la Orbita 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). These conditions are not cancer. Eyelid contour deformity due to lachrymal gland enlargement both orbits.red Arrows show the actual tumors within the orbits as seen on a CT scan. Orbital inflammatory conditions can affect any given orbital structure: Lacrimal gland, eyelids, eye moving muscles, orbital fat, or the orbit as a whole. All-too-often a well-defined mass is present in the orbit, though orbital pseudotumor can also be an inflammatory process with poorly defined margins and borders, a rather diffuse orbital infiltration that increases orbital volume as a whole but lacks a well-defined mass. All too often, a chronic eyelid swelling that does not go away is due to an underlying orbital inflammatory condition. Orbital pseudotumor can often be quite painful. In addition to pain, an inflammatory mass (tumor) can make the patient's eye bulge out (proptosis) and restrict the movement of the eye. A biopsy (surgery) is commonly performed to confirm the diagnosis of orbital pseudotumor and rule out other diseases that cause orbital inflammation. 1 / 5

2 Symptoms may vary depending on the location of the inflammatory mass itself. An inflammation affecting eye muscles would cause pain on eye movements and double vision. Lachrymal gland affection could cause a dry eye, eye-surface-discomfort and foreign body sensation, as well as superior eyelid contour deformity and soreness to light touch. Orbital inflammatory disease is typically characterized by the rapid development of pain or soreness, proptosis (bulging eye), 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. True defined masses (tumors) are not usually present, rather a diffuse orbital volume increase its all that is found. 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 can be treated without a biopsy. These cases typically respond rapidly to steroid therapy (which helps confirm the diagnosis). Atypical or suspicious cases of orbital pseudotumor usually undergo biopsy, which helps establish the diagnosis and rule out lymphomas or different autoimmune inflammatory process. 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. 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. Orbit 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. 2 / 5

3 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 are: - Thyroid Eye Disease - Sarcoidosis - Infectious Orbital Cellulitis - Orbital Myositis - Scleritis - Orbital Vasculitis - Sjogren's Disease - Wegener's Granulomatosis - Malignant Ocular Tumors Sclerosing Orbital Pseudotumor 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. 3 / 5

4 A. Shows a massively infiltrated medial rectus muscle B. and causes performed history sinus Sclerosing with clinical mass. confirmed Shows cavernous combinations disease of picture should sinusitis, a to orbital diffuse by rule should and be sinus. pathology, out pseudotumors performed. of sinus the inflammatory surgery, be Wegener's patient's addressed. surgery, systemic For steroid is response infiltration are Granulomatosis. or example, usually testing inhalation less therapy, responsive diagnosed that treatment. rule radiation drug ANCA occupies Many out abuse. by to blood specific CT and steroid biospy of the these scan Therefore, test chemotherapy deep infectious therapy. (orbitotomy). and of patients orbital a concurrent chest Most and vertex, have depending shows Once x-ray inflammatory cases a optic past treatable should the an are orbital medical upon diagnosis nerve, treated bethe Lymphoid Tumors Lymphoid tumors are one of the more common orbital tumors despite the orbit not containing lymph nodes or a well-defined lymphatic vasculature.the incidence is between 4 and 13% of all orbital tumors. Orbital lymphomas may be primary or associated with systemic disease. Although most orbital lymphomas are localized to the orbit at diagnosis, many patients will develop systemic lymphoma over time. Approximately 20% of patients with lymphoid tumors of the conjunctiva, 35% of patients with orbital tumors and 67% with eyelid disease will eventually develop systemic lymphoma. Orbital lymphoma is an adult disease process usually presenting between the age of 50 and 70 years. The course is usually one of an anterior mass which enlarges slowly causing progressive painless proptosis over several weeks to months. The classic lesion is a smooth, pink-orange 4 / 5

5 mass ("salmon patch") under an intact conjunctiva. CT scan shows a homogeneous mass with well-defined borders that does not destroy surrounding structures or bone. Most lesions are extraconal and in the superior orbit. The lacrimal gland may be affected but enlarges with a normal shape unlike primary lacrimal gland tumors, which distorts normal gland shape as they grow. To make a definitive diagnosis a generous biopsy is needed and must be sent for immunohistochemical studies (fresh) as well as permanent section (formalin). Once a diagnosis of lymphoma is made, patients should be worked up for systemic disease including a complete physical exam, a complete blood count, bone marrow biopsy and CT scans of the thorax, abdomen and pelvis. Patients with localized lymphoma to the orbit may be treated with primary XRT, and patients with systemic disease may respond to a combination of orbital radiotherapy and systemic chemotherapy. Consultation with an oncologist should be obtained. Left. An orbital infiltrative lesion is present in the right orbit. Patient presents with deep orbit discomfort, moderate proptosis (eye bulging), and foreign body sensation in the eye due to dry eye. Right. A thickened eye muscle due to lymphoma infiltration. 5 / 5