13/01/2025
CASE ANALYSIS
Male patient 70 y/o come to office by blur vision OD and long standing data Orbital tumor ; patient was examined by ophtalmology MD , who he’s not recommended orbital surgery by compromise orbital structures .
VA:
OD: SC: 20/40
OS: SC : NPPL
EOMs: over acc LLR, hypo acc LMR
DIAGNOSIS : LEFT ORBITAL LYMPHOMA PROLIFERATIVE
Orbital lymphoma is a type of non-Hodgkin lymphoma (NHL) that originates in the conjunctiva, lacrimal gland, soft tissues of the eyelid, or extraocular muscles; it is most commonly extraconal in location. Orbital lymphoma is said to be primary when it arises spontaneously from one of these locations, and secondary when it is associated with metastatic spread from an extraorbital site.
Epidemiology
While lymphoma constitutes more than half of all orbital malignancies (55 percent),1 the incidence of orbital lymphoma has been reported to account for between 1 and 10 percent of NHL cases.2
Orbital lymphoma may be unilateral or bilateral. Although it has been known to present in patients between 15 and 70 years of age, most cases cluster around the seventh decade. Historically, a female preponderance has been noted,3 but there have been several conflicting reports regarding gender predominance. Geographically, the disease is most common in Asia and Europe.
Pathophysiology
Immunosuppression due to any cause—including AIDS, immunosuppressive drugs, or increasing age—has long been established as the main factor contributing to the pathophysiology of orbital lymphoma.
More recently, however, the role of various pathogens has entered into the debate. These include associations with Chlamydia psittaci and Helicobacter pylori, as well as some viruses.
Histology
Almost 80 percent of orbital and adnexal lymphomas are of low-grade variety, with B-cell lymphomas and extranodal marginal zone lymphoma of the mucosa-associated lymphoid tissue type (MALT lymphoma) being the most common histological diagnoses.4 MALT lymphomas comprise a distinct set of tumors that exhibit the unique property of “homing,” which permits lymphoma cells to adhere to other epithelial and mucosal sites, thus enabling bilateral involvement.
Other subtypes have also been reported, including follicular lymphomas, diffuse large B-cell lymphomas, and mantle cell lymphomas.
Clinical Presentation
The clinical presentation is generally nonspecific and depends on the location of the lymphoma. Vision is usually preserved, as infiltration of the globe or optic nerve is rare.
Conjunctiva. Patients typically demonstrate a pink or red “salmon patch” of swollen conjunctiva or conjunctival hyperemia.
Orbit. Orbital presentation is most commonly observed as a painless palpable mass in the superolateral quadrant. It may lead to proptosis, ptosis, diplopia, or abnormal ocular movement.
Lacrimal gland. The enlarged gland displaces the eyeball inferomedially.
Eyelids. Swelling and prolapse may occur in the eyelid.
Diagnosis
The differential diagnosis of orbital lymphoma includes the following:
Pseudotumor
Orbital metastases
Diffuse lymphangioma
Lacrimal adenoma
Cavernous hemangioma
Lymphoid lesions of the orbit (benign reactive lymphoid hyperplasia, atypical lymphoid hyperplasia)
Diagnostic workup. The following evaluations form the basis of a good diagnostic workup, undertaken in conjunction with the patient’s internist:
History and physical examination
Dilated fundus examination
Thorough examination of opposite orbit as well as the oral cavity and oropharynx
Complete blood count, biochemistry profile
Fine-needle aspiration and biopsy
Liver function tests, renal function tests
Chest x-ray
Computed tomography (CT) and magnetic resonance imaging (MRI) of orbit, abdomen, thorax, and pelvis
Bone marrow aspiration
Imaging
Computed tomography. The typical finding on CT scanning is a moderately well-defined orbital mass, which molds to the adjacent ocular structures without bony destruction. It is usually homogeneous in density, either isodense or slightly hyperdense when compared with the density of the extraocular muscles. It may exhibit any of the following four patterns: retroocular, anterior preseptal, lacrimal gland involvement, or extension of an adnexal lesion.
Lesions that are heterogeneous, with bony destruction, are indicative of high-grade lymphomas, which are usually accompanied by pain.
Magnetic resonance imaging. Compared with the extraocular muscles, the orbital mass appears isointense or hypointense on a T1-weighted MRI scan, but it appears isointense to hyperintense on a T2-weighted scan. Hypointense T2-weighted images are suggestive of high-grade lymphomas.
Contrast-enhanced imaging techniques. Contrast-enhanced CT/MRI scans reveal mild to moderate enhancement of the lesion, and when this is seen in T1-weighted images, high cellularity of the lymphoma can be expected. High cellularity in lymphoma produces a lower apparent diffusion coefficient (ADC) of