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06-16-2012, 08:04 PM
Orbital Lymphoma:

60 year-old male who was evaluated 1 wk ago for "conjunctivitis" OS by internal medicine

Andrew Doan, MD, PhD, Thomas Oetting, MD
February 21, 2005
Chief Complaint: 60 year-old male who was evaluated 1 wk ago for "conjunctivitis" OS by internal medicine.
History of Present Illness: Patient complained of a painless, gradual redness of his left eye over the last several months. He does not report any pain, discharge, discomfort, or itching. He presented to the on-call ophthalmologist for evaluation. He pointed to some fullness of the superior aspect of the medial canthus OS with mild conjunctival injection. Pt also complained that things were "blurry" with a slight double image, which resolved when one eye was closed separately.
PMH: h/o non-Hodkin's lymphoma and in remission for numerous years. No other ocular history or medical problems. No h/o of ocular traumas or surgeries.
EXAM

Best corrected visual acuities: 20/20 OU.
Pupils: 4.5->3.5, no RAPD
EOM: elevation deficit OS with left hypotropia in primary gaze; VF Full to FC OU
IOP: 18 mmHg OD, 19 mmHg OS
DFE: retina exam notable for normal macula, vessels, and periphery OU. Optic nerves, normal.
SLE: OD normal, OS see photos below.
Hertel (base 113): 13 mm OD and 17 mm OS
Figure 1: EOM- Note the left hypotropia in primary gaze and elevation deficit OS with upgaze.
http://webeye.ophth.uiowa.edu/eyeforum/cases-i/cases/EOM_03222004.jpg
Figure 2: Left eye with superior conjunctival injection.
http://webeye.ophth.uiowa.edu/eyeforum/cases-i/cases/OS_03222004.jpg
Figure 3: Left medial canthus with fullness. Note the asymmetric ptosis OS (more ptosis medially).
http://webeye.ophth.uiowa.edu/eyeforum/cases-i/cases/OS2_03222004.jpg
Figure 4: CT ScanCoronal CT without contrast.

Axial CT without contrast.

Note: large medial mass indenting globeNote: large medial mass displacing medial rectus but is not eroding into bone or surrounding soft tissue. http://webeye.ophth.uiowa.edu/eyeforum/cases-i/cases/CT_03222004.jpghttp://webeye.ophth.uiowa.edu/eyeforum/cases-i/cases/CT2_03222004.jpg
Figure 5: Anterior orbitotomy. Note the fleshy mass in the superior-nasal quadrant.
http://webeye.ophth.uiowa.edu/eyeforum/cases-i/cases/orbitotomy_03222004.jpg
Discussion

Orbital Lymphoma

This patient presented with acute awareness of a long-standing problem: fullness of upper lid, binocular diplopia, and injection OS. He was diagnosed previously with "conjunctivitis" because of the asymmetric injection OS; however, he did not have other signs of infection: pain, itching, tearing, or discharge. On exam, his asymmetric ptosis OS (i.e. mechanical ptosis), elevation deficit OS on versions, proptosis OS, and a firm palpable mass in the superior-nasal quadrant OS should raise the suspicion of an orbital mass. In a patient with a history of non-Hodkin's lymphoma, orbital spread needs to be ruled out. The CT scans clearly denote a large orbital mass that is molding surrounding structures but not eroding into them.
An anterior orbitotomy was notable for a large, pink mass. Pathology confirmed the mass to be positive CD20 and BCL-2 lymphoma. Extensive imaging studies of head and body did not reveal additional tumor. The patient was treated by radiation oncology with local radiation.
Diagnosis: Orbital Lymphoma

EPIDEMIOLOGY


Incidence: in Florida, 2 cases per million
Age: all ages
Among the most common orbital tumors seen in the adults: cavernous hemangioma, lymphoid tumors, and meningiomas
SIGNS


Salmon patch lesion on globe
CT scan will show a lesion that pushes and molds surrounding structures and bone. There is no erosion into surrounding tissue.
Non-tender, firm mass
SYMPTOMS


Gradual onset
Painless
Slow progression
Binocular diplopia
Extraocular motility problems
Proptosis
Mechanical ptosis from tumor pushing down lid
TREATMENT


Depends on type of tumor and extension.
Chest and abdominal CT are used to identify abnormal nodes and spread.
Bone marrow aspiration to rule out marrow involvment.
If there is no systemic involvement, orbital lymphomas are typically sensitive to local radiation.
Chemotherapy is an option for patients with systemic involvment.
Incisional biopsy is needed for pathologic diagnosis.

Differential Diagnoses

lymphoid lesions of the orbit (benign reactive lymphoid hyperplasia, lymphoma, atypical lymphoid hyperplasia)
cavernous hemangioma
optic nerve meningioma
orbital metastasis
neurofibroma
neurilemoma (a.k.a. benign schwannoma)
fibrous histiocytoma
hemangiopericytoma
lymphangioma
mucocele
thyroid disease (most common cause of unilateral proptosis in adults)
References


Hasegawa M, Kojima M, Shioya M, Tamaki Y, Saitoh J, Sakurai H, Kitamoto Y, Suzuki Y, Niibe H, Nakano T. Treatment results of radiotherapy for malignant lymphoma of the orbit and histopathologic review according to the WHO classification.Int J Radiat Oncol Biol Phys. 2003 Sep 1;57(1):172-6.
Margo CE, Mulla ZD. Malignant tumors of the orbit. Analysis of the Florida Cancer Registry.Ophthalmology. 1998 Jan;105(1):185-90.
Nerad, JA. Oculoplastic Surgery. The Requisites in Ophthalmology. Mosby 2001. 348-386.