Orbital Lymphoma - University of Louisville Department of
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Transcript Orbital Lymphoma - University of Louisville Department of
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
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
Right orbitotomy with bone flap for lacrimal
gland excisional biopsy
Right nasolabial fold mass excision
Pathology
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
Pt doing well
VA OD improved slightly from 20/70 to 20/50
Release back to Dr. Woodcock for further workup/therapy
Orbital Lymphoma
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
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
Labortatory studies
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)
MRI
Decreased ability to evaluate for bony destruction
May miss conjunctival disease
T1: isointense
T2: hyperintense
PET
Best to check for systemic/nodal involvement
Higher sensitivity than CT (86% vs. 72%)
Biopsy and Histopathology
Essential to confirm diagnosis
Histology and immunophenotype
Grading
Monoclonal------>Polyclonal
20%--------->60% risk of systemic disease
Treatment
Radiotherapy is the treatment of choice (97-100% local control)
Surgery is usually NOT recommended due to their infiltrative
nature
Systemic disease
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
Every 6 months for 2 years with repeat imaging
Annually thereafter
Prognosis
Decreases with systemic involvement and/or bilateral
disease
Major factors
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
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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