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Differentiating Orbital Tumors in the Aging Population. The case of 66 - year old man with recurrent red left eye and edema of lower left eye lid led to the diagnosis of Non Hodgkin’s Lymphoma: an orbital tumor arising from from mucosaassociated lymphoid tissue of ocular adnexa. 1. Case History - Patient demographics: 66 – year old Caucasian male - Chief Complaint: This patient presents on 08/03/15 with recurrent red left eye and tearing, patient states he has been having this problem for the past one year and was treated with some topical antibiotics but the problem has not resolved. The patient was seen 25 days prior at which time a diagnosis of bacterial conjunctivitis was conferred based on clinical observations. The patient states he had double vision some time ago but does not have any currently and reports some mild aches every now and then in the left eye. - Ocular, medical history: i. Ocular 1. Recent diagnosis of bacterial conjunctivitis in the left eye, he was instructed to use erythromycin ointment BID in the left eye, patient used it for only 10 days and self discontinued, he states it was not helping with the redness. 2. Exudative Age Related Macular Degeneration OU; patient is legally blind due to end stage disease, he has had multiple Avastin/Lucentis injections OU. 3. History of Non-arteritic ischemic optic neuropathy OD IN 2007 4. Xanthelasma OD. ii. Medical 1. 2. 3. 4. 5. - Non-insulin dependent diabetes mellitus Hypertension Coronary artery disease Transient cerebral ischemia Obesity. Medications: i. Systemic 1. Albuterol spray inhaler/oral 2. Glipizide, 3. Insulin injection 4. 5. 6. 7. 8. 9. Ibuprofen Lisinopril Metformin Metoprolol Simvastatin Zoster vaccine injection. ii Ocular: none - Pertinent Findings: 1. Visual acuity: 20/200 OD, PH no improvement 20/400 OS, PH no improvement 2. Pupils: OU: PERRL 3. CF: OU: full 4. EOM: OD: Full range OS: Slight restriction inferior, negative pain, negative diplopia 5. Anterior segment: Adnexa: OD: normal OS: mass inferior lower lid area into left adnexa/upper maxillary area, firm to hard on palpation and mild erythema. Lids/Lashes: OD: mild mild scurf lower lid, mild meibomian gland stasis. OS: mild erythema, small papilloma with dilated vessels lower lid, mild meibomian gland stasis. Palpebral conjunctiva: OD: concretions OS: concretions, engorged blood vessels deep inferior fornix. Bulbar conjunctiva: OD: trace diffuse injection OS: grade 1-2 diffuse injection, Cornea: OD: clear OS: inferior superficial punctate keratitis. Anterior chamber: OU: deep and quiet Angle: OU: 3 Iris: OU: clear and flat Orbit. OD: negative proptosis OS: Mild proptosis 6. Goldman intraocular pressure: 15/15 @1350 Dilated fundus examination: lens: OU: 1+ NS/gr1 ACC C/D: OD: 0.35 v/h OS: 0.20 v/h ONH: OD: distinct margins, (+) pallor OS: distinct margins, (-) pallor vessels: OU: mild attenuation macula: OU: elevated 3DD disciform scar with scattered pigment clumping. Pos-Pol: OU: fibrosis in the superior arcade, periphery: OD: chorio-retinal scar sup/nasal OS: chorioretinal scar sup/temp Vitreous: clear OU 7. Radiology Studies: CT scan of orbits with and without contrast 08/04/15: OD: Unremarkable OS: A large lobulated extra-conal is seen at the inferior aspect of the left orbit which extends through the orbital floor in the region of the left infraorbital foramen. This mass extends into the anterior portion of the left maxillary sinus and through the anterior wall of the left maxillary sinus. There is no mass in the ethmoid region, the ostiomeatal units are patent and the mass effect caused by this lesion threatens the left globe , while there is no direct invasion of the globe or rectus muscles. MRI of the orbit with and without contrast shows irregular mass , hypointense to fat and hyper intense to muscle. Biopsy of the mass shows B cell lymphoma. PET scan shows primary B cell lymphoma with no systemic associated lymphoma. 8. Treatment and Management:Patient was referred to an orbital tumor specialist who performed a tissue biopsy, the result of the biopsy showed primary B cell lymphoma of the left orbit.Patient is currently under the care of the orbital tumor specialist and will be started chemotherapy treatment. Discussion: Primary Lymphoma of the orbital lymphoma is one of the commonest orbital tumors. It is a Bcell non Hodgkin lymphoma and in commonly arises from mucosa- associated lymphoid tissue. Orbital lymphoma accounts for a very small percentage of all lymphomas but constitutes approximately half of all primary orbital malignancies in the adult population. The tumor typically occurs in the 5th -7th decades of life with no gender predilection. This tumor typically presents as a palpable firm and its clinical presentation can be variable with about a quarter of the patients presenting with injection and chemosis. This tumor is usually located in the superior lateral quadrant of the orbit but can be found less in other quadrants. It can also present with exophthalmos, ptosis and some patients might complain of diplopia and abnormal ocular movement. The mass is generally painless but some patients may show inflammatory-like changes with pain, erythema and edema. It rarely infiltrates the optic nerve and vision is rarely affected. This tumor is differentiated from other orbital masses by the non involvement of the extraocular muscles as tits origin. CT Scan without contrast of the tumor is usually homogenous in density when compared to the extra ocular muscles. CT scan with contrast shows only moderate enhancement. MRI of the tumor shows an isointense signal to muscle on T1- weighted images and isohyperintense to muscle on T2-weighted images. Treatment of Orbital lymphoma involves surgical biopsy/resection, radiotherapy and chemotherapy. There might be a relapse free 5 –year rate in approximately 65% of cases with less than 5% of patients eventually dying from the disease. Differential Diagnosis: a.Orbital rhabdomyosarcoma: This tumor typically occurs in children below the age of 15 years and presents with rapidly enlarging mass. It usually presents with proptosis and diplopia but is usually painless. CT scan is typically homogenous soft tissue masses isodense to normal muscle. MRI T1 shows low to intermediate intensity, isointense to adjacent muscle and MRI T2 is usually hyperintense. b. Orbital Pseudotumor: This is an idiopathic inflammatory condition that usually involves the extra ocular muscles and in some cases there is inflammatory change involving the uvea, sclera, lacrimal gland and retrobulbar tissues.Patients typically present with unilateral proptosis and diplopia of rapid onset. These typically resolve in most cases with steroid treatment although more chronic progression may require chemotherapy and radiotherapy.CT scan shows enlargement of the muscle belly of one or ocular muscles and involvement of the tendinous insertion. MRI T1 shows iso to hypo intense of affected region. MRI T2 affected region hypo intense secondary to fibrosis and hyper intense as fibrosis progresses. c. Orbital metastases: Extra ocular orbital metastases are relatively uncommon and in general patients with metastatic disease do not develop orbital metastasis. Conclusion: Orbital lymphoma is diagnosed by CT Scan and MRI. Bibliography: 1. Kanski JJ. “Tumor”.198-203. Clinical Ophthalmolgy: 6th Edition. ButterworthHeinemann.2007 2. Ehlers.P. Justus, Shah .P. Chirag. “Orbital Tumors”. 156-166 .The Wills Eye Manual. 5th Edition. 3. Kaiser, Friedman, Pineda. “Adult Orbital Tumors”. 21-23. 2nd Edition. 4. Shields JA, Shields CL, Scartozzi R. Surveyof 1264 patients with orbital tumors and simulating lesions: the 2002 Montgomery Lecture, part 1. Ophthalmology 2004;111(5):997–1008. CrossRef, Medline 5. Lowe LH, Marchant TC, Rivard DC, Scherbel AJ. Vascular malformations: classification and terminology the radiologist needs to know. Semin Roentgenol 2012;47(2):106–117. CrossRef,Medline 6. Ruchman MC, Flanagan J. Cavernous hemangiomas of the orbit. Ophthalmology 1983;90(11): 1328–1336. CrossRef, Medline