Orbital Lymphoma - University of Louisville Department of

Report
Grand Rounds Conference
Eric Downing MD
University of Louisville
Department of Ophthalmology and Visual Sciences
Subjective
CC/HPI: 77M referred by a local oncologist for a
painless “bump” on RUL for 2 months. Pt had
similar bumps on LUL in 2006, which were
excised and identified as benign, per pt.
History
POH: lump removed over left eye, CEIOL OU,
non-ischemic CRVO
PMH: Throat cancer (2003) treated with 6 rounds
of chemotherapy, multiple kidney stones
(2012)TIA (2008), HTN
Eye Meds: artificial tears
Meds: Norvasc, ASA, Ativan
Objective
VA:
Pupils:
IOP:
EOM:
OD
20/70
4
19
full
OS
20/20
4
21
full
Objective
SLE:
E/L/L: Superotemporal mass under RUL, second mass
under right nasolabial fold
C/S: White, quiet OU
K:
Clear OU
AC: Deep and quiet OU
I/L: Round, PCIOL OU
Hertel 18, 18
Differential diagnosis
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Benign lymphoproliferative disorder
Metastatic lesion
Inflammatory lesion
Infectious lesion
Epithelial tumor
Assessment

57M with history of throat cancer (2003), treated
with chemotherapy, previously in remission,
who presented with a right superotemporal
orbital mass for two months.
Plan


MRI with gadolinium
Excision of masses with biopsy
MRI
T1
T2
Surgery

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Right orbitotomy with bone flap for lacrimal
gland excisional biopsy
Right nasolabial fold mass excision
Pathology
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Biopsy: Follicular lymphoma, grade 3A
CD 10, CD 20, BCL-2, and BCL-6 positive
Ki67 positive in 30-40% of cells
CD10, BCL-2: Follicular
CD10/19/20/22/23: B cell
Ki67 is a cell proliferation marker
CD2/3/4/5/7/8/56: T-cell lymphoma
Post-op
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Pt doing well
VA OD improved slightly from 20/70 to 20/50
Release back to Dr. Woodcock for further workup/therapy
Orbital Lymphoma
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Orbital lymphomas account for 8-15% of extranodal
non-Hodgkins lymphomas
MALT lymphomas are the most common (~50%),
followed by the follicular type (~25%)
Systemic involvement in approx 1/3 of cases
5 year survival rate approaches 100% for follicular
lacrimal gland tumors, and 70% overall for extranodal
marginal zone tumors
Divided into 3 grades which refer to the number of
centroblasts per HPF
Pathophysiology



Progressive clonal expansion of B, T, or NK
cells due to mutations affecting the protooncogenes or tumor-suppressor genes
Majority are non-Hodgkin B-cell lymphomas
(90%)
Often associated with infectious entities, such as
Chlamydia psittaci
Epidemiology
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Lifetime risk for NHL is 2%
Typically affects elderly patients, with mean ages in
the seventh decade
Slight female predominance
Asians/Pacific Islanders>whites>blacks
Increases of >6% annually between 1975-2001
Increasing incidence, most likely due to better
diagnostic techniques, the aging population,
increased use of immunosuppressive drugs, and
HIV/AIDS
History & Physical
Painless proptosis with or without
motility deficits
Ptosis
Rarely have decreased VA
If conjunctival, can have the
characteristic salmon-patch appearance
Cervical or preauricular
lymphadenopathy, parotid gland swelling,
or abdominal mass can portend systemic
disease

Work-up
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Labortatory studies
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CBC, RPR, ESR, LDH, FTA-Abs, HIV
Lumbar puncture
Imaging
CT: seen as well-defined, high density, lobulated or
nodular masses with sharp margins. Usually no
bony erosion.
 Usually extraconal
 10-17% involve the lacrima

Imaging (con’t)
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MRI
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Decreased ability to evaluate for bony destruction
May miss conjunctival disease
T1: isointense
T2: hyperintense
PET
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Best to check for systemic/nodal involvement
Higher sensitivity than CT (86% vs. 72%)
Biopsy and Histopathology
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Essential to confirm diagnosis
Histology and immunophenotype
Grading
Monoclonal------>Polyclonal
20%--------->60% risk of systemic disease
Treatment
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Radiotherapy is the treatment of choice (97-100% local control)
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Surgery is usually NOT recommended due to their infiltrative
nature
Systemic disease
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Dose of 20-40 cGy
Low-grade generally refractory to chemotherapy, but often have a long survival
rate, even if untreated
Aggressive lesions are treated with radiation and chemotherapy—often
Methotrexate +/- procarbazine, vincristine, thiotepa
Intralesional Rituximab for low-grade lymphomas (Savino)
Complications of radiotherapy: conjunctivitis, cataract, dry eye, corneal
ulcer, NVG, optic neuropathy
Follow up
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Every 6 months for 2 years with repeat imaging
Annually thereafter
Prognosis
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Decreases with systemic involvement and/or bilateral
disease
Major factors
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Anatomic location
Stage at presentation
Subtypes (ENMZ(0-20%)>FL(20-37%)>MCL(38100%>LPL(14-100%)
Immunohistochemical markers
20-25% will develop disseminated disease within 5
years
ENMZ: extranodal marginal zone, FL: follicular, MCL: mantle cell, LPL: lymphoplasmatic
Research
Analyzed scans of 23 patients with either orbital
lymphoma or IgG4 disease
 All patients underwent both MRI and Diffusionsensitised driven-equilibrium prep (DSDE)
 Used an apparent diffusion coefficient to
differentiate
 Lymphoma has a lower ADC than IgG4 tissue,
appearing darker, and giving us an additional
modality to differentiate the two

MRI images
DSDE images
Lymphoma
IgG4 disease
References
1.
2.
3.
4.
5.
BCSC: Orbit, Eyelids, and Lacrimal System. Pp79-84
Rasmussen P, Ralfkiaer E, Prause JU, et al. Malignant Lymphoma of the
Lacrimal Gland. Arch Ophth. 2011 Oct. Vol 129(10):1275-1280.
Eckardt et al. Orbital Lymphoma: diagnostic approach and treatment
outcome. World Journal of Surgical Oncology. 2013. 11:17
Savino G, Battendieri R, Gari M, et al. Long-term outcomes of primary
ocular adnexal lymphoa treatment with intraorbital rituximab injections. J
Cancer Res Clin Oncol. 2013; 139(7):1251-5
Hiwatashi A, Yoshiura T, Togao O. Diffusivity of intraorbital lymphoma vs
IgG4 related disease: 3D turbo field echo with diffusion-sensitised driveequilibrium preparation technique. Eur Radiol. 2014, 24:581-586

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