Download Differentiating Orbital Tumors in the Aging Population. The case of

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.
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lesions: the 2002 Montgomery Lecture, part 1. Ophthalmology 2004;111(5):997–1008.
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terminology the radiologist needs to know. Semin Roentgenol 2012;47(2):106–117.
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