A Woman with Decreased Vision and Diplopia

Posted by • June 12th, 2015

In the latest Case Record of the Massachusetts General Hospital, a 41-year-old woman presented with decreased visual acuity in the left eye and diplopia. MRI of the head and orbits revealed abnormal soft tissue in the left sphenoid sinus and orbital apex, extending to the left cavernous sinus. A diagnostic procedure was performed.

Lymphoma of the orbit is typically painless and has an indolent course, and thus the presence of pain and subacute progression of symptoms may suggest a different diagnosis or a more aggressive type of lymphoma.

Clinical Pearls

What conditions may predispose to the development of orbital cellulitis?

Orbital cellulitis commonly results from bacterial infection, most often as an extension of ethmoid or frontal sinusitis, but it may also result from cutaneous trauma, dental abscess, or dacryocystitis.

The organisms most commonly associated with orbital cellulitis are streptococcal and staphylococcal species.

What diseases are included in the differential diagnosis of an inflammatory process involving the orbit?

Inflammatory disease of the orbit is common, and causes include idiopathic orbital inflammation, IgG4-related orbital inflammation, sarcoidosis, granulomatosis with polyangiitis, and proliferative disorders of histiocytes. Idiopathic orbital inflammation, which is by far the most common of these diseases, was previously known as orbital pseudotumor and refers to inflammation involving any structure of the orbit. Specific descriptive nomenclature includes dacryoadenitis, scleritis, and myositis, although many cases involve diffuse infiltration of the orbital fat. This usually painful condition often results in visible periorbital inflammation and may occasionally extend to involve the paranasal sinuses or dura. The presence of pain is often clinically useful in making the diagnosis, but the absence of pain can be misleading. IgG4-related disease is an orbital inflammatory disorder that is less common than idiopathic orbital inflammation. Patients with IgG4-related disease have clinical and radiographic presentations that are similar to those of patients with idiopathic orbital inflammation, but IgG4-related disease is more likely to be bilateral and associated with an inflammatory disorder of another organ system. Sarcoidosis is a granulomatous disease that may involve the lungs, liver, spleen, eyes, and orbit. Orbital sarcoidosis most often involves the lacrimal glands but may involve other orbital structures and extend through apical foramina to the surrounding structures.

Morning Report Questions

Q: What clinical and imaging features characterize lymphoid tumors involving the orbit?

A: Lymphoid tumors are common infiltrative orbital cancers and range from the most common variety, indolent mucosa-associated lymphoid-tissue lymphomas, to more rare, aggressive varieties.

Lymphoma may involve any orbital structure — commonly including the lacrimal gland, extraocular muscle, or fat — and may be part of a systemic process. B-cell lymphomas are the most common type to involve the orbit and tend to be unilateral, painless, and slow-growing. On radiography, lymphoma has an infiltrative pattern, with molding to the surrounding structures.

Q: Is CD30 expression a common feature of diffuse large B-cell lymphoma?

A: Diffuse large B-cell lymphoma represents a group of biologically heterogeneous cancers that may be divided into morphologic, genetic, and immunophenotypic subgroups and that include certain specific disease entities. Most cases do not fulfill diagnostic criteria for one of the specific disease entities and are classified as diffuse large B-cell lymphoma (not otherwise specified). CD30 expression is seen in only 14% of cases of diffuse large B-cell lymphoma, and CD30-positive cases have been reported to be associated with a superior 5-year overall and progression-free survival, as compared with CD30-negative cases, a difference that is maintained in both germinal-center and nongerminal-center subgroups. Gene-expression profiling studies have shown a distinct profile, suggesting that CD30-positive cases may represent a distinct subgroup of diffuse large B-cell lymphoma.

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