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CASE REPORT

Epibulbar Plasmacytoma Masquerading as Subconjunctival


Hemorrhage in a Patient With Multiple Myeloma
Amanda Bradley, BS,* Amy Estes, MD,*† Lane Ulrich, MD,*† Dilip Thomas, MD,*† and David Gay, MD*†

is conventionally a disease of older adults, with the median


Purpose: We report a 75-year-old woman with a history of multiple age of diagnosis being 66 years. African Americans are twice
myeloma immunoglobulin D (IgD) variant, who presented with an as likely as whites to be affected by this disease.
epibulbar plasmacytoma masquerading as a subconjunctival hemorrhage. Multiple myeloma is characterized by proliferation of
Methods: Magnetic resonance imaging of the brain and orbits with plasma cells in the bone marrow, which typically results in
and without contrast was obtained and surgical biopsy of the widespread skeletal destruction as a result of osteolytic
subconjunctival lesion was performed; histopathology confirmed the lesions and associated pathologic fractures. Bone pain is
diagnosis of plasmacytoma. the most common clinical presentation; however, other
hallmarks of the disease include anemia, recurrent infec-
Results: Subconjunctival biopsy revealed a plasma cell neoplasm tions, hypercalcemia, spinal cord compression, and renal
infiltrate in the episcleral layer. The subconjunctival biopsy stained insufficiency.2
positive for CD138 and lambda-immunohistochemistry in the majority Although rare, multiple myeloma may manifest with
of plasma cells. Histologic findings were consistent with involvement involvement in virtually any ocular structure. A few of the
by known IgD plasma cell myeloma where previous bone marrow commonly reported ocular findings include orbital plasmacy-
biopsy demonstrated myeloma cells which stained monoclonally for tomas, ciliary body cysts, exudative macular detachment,
IgD-lambda light chains. copper deposition in the cornea, retinal hemorrhages, and
cotton wool spots.3 Ocular pathology may occur as a result of
Conclusions: Although plasma cell neoplasms seldom present with direct infiltration of malignant plasma cells, displacement of
ocular manifestations, it is crucial to recognize that these tumors may ocular tissues by plasmacytomas, or hematologic derange-
be associated with multiple myeloma. In patients with known multiple ments with increased plasma viscosity or protein deposition in
myeloma who present with subconjunctival hemorrhage, close follow- tissues. To the best of our knowledge, this is the only case of
up is highly recommended, as this may be the initial presentation of an subconjunctival plasmacytoma initially disguised as
ocular plasmacytoma. Although a plasmacytoma is a rare subcon- subconjunctival hemorrhage.
junctival lesion, it should not be immediately excluded from the
differential diagnosis of such lesions.
Key Words: multiple myeloma, plasmacytoma, subconjunctival CASE REPORT
hemorrhage A 75-year-old woman with known refractory immunoglobulin D
(IgD) multiple myeloma was referred to the Ophthalmology Depart-
(Cornea 2017;36:249–251) ment at Augusta University for evaluation of new-onset subconjunctival
hemorrhage. She reported mildly blurred vision with bulging of her left
eye and associated redness. She was receiving chemotherapy and had
recently had administered warfarin. She denied any recent trauma or
M ultiple myeloma is a treatable yet incurable malignancy,
which presents as monoclonal proliferation of abnormal
plasma cells, resulting in overproduction of a clone of
excessive rubbing. Slit-lamp examination of the conjunctiva revealed
diffuse subconjunctival hemorrhage, most prominent at the nasal bulbar
conjunctiva OS, resulting in delle formation. She received conservative
immunoglobulins. This plasma cell dyscrasia accounts for therapy with erythromycin ointment and artificial tears. At follow-up,
approximately 10% of all hematologic malignances. The age- ocular examination of the conjunctiva and the sclera demonstrated
adjusted incidence of multiple myeloma is 6.56 cases per resolving subconjunctival hemorrhage. Evaluation of the cornea was
100,000 persons per year in the United States according to the significant for resolution of the delle.
National Cancer Institute’s Surveillance Epidemiology and Four months later, the patient returned with complaints of
End Result (SEER) data released in 2012.1 Multiple myeloma a 2-week history of recurrent subconjunctival hemorrhage in the
same location as the previous hemorrhage OS. Examination of the
conjunctiva and the sclera of her left eye demonstrated elevation of
Received for publication July 8, 2016; revision received September 19, 2016; the nasal bulbar conjunctiva with an erythematous and cystic
accepted September 20, 2016. Published online ahead of print November subconjunctival mass present in the location of the previous
16, 2016. subconjunctival hemorrhage (Fig. 1). A foamy epithelial lesion,
From the *Medical College of Georgia, Augusta University, Augusta, GA; and
†Department of Ophthalmology, Augusta University, Augusta, GA. continuous with a subconjunctival mass extending 2 mm onto the
The authors have no funding or conflicts of interest to disclose. cornea, was noted on inspection. The remainder of her anterior
Reprints: Amy Estes, MD, Department of Ophthalmology, Augusta Univer- segment was unremarkable.
sity, 1120 15th St, Augusta, GA 30912 (e-mail: aestes@augusta.edu). Although atypical in size and location, the initial clinical
Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved. concern was for a plasmacytoma, and magnetic resonance imaging

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Bradley et al Cornea  Volume 36, Number 2, February 2017

FIGURE 1. Clinical appearance of


a subconjunctival mass on return
appointment in January 2016.

(MRI) was performed to rule out orbital involvement. MRI with DISCUSSION
and without contrast of the brain and orbits showed a left medial Multiple myeloma is malignant proliferation of plasma
canthal episcleral soft tissue mass measuring 7.4 · 10.1 · cells in the bone marrow. Plasma cells are the most mature
14.6 mm in AP, transverse, and vertical diameters on axial
T2-weighted series (Fig. 2). The lesion demonstrated predomi-
form of B lymphocytes and are responsible for encoding the
nantly hypointense behavior with positive diffusion signal arrangement of mature immunoglobulins. In multiple mye-
abnormality concerning for high cellular packing. MRI demon- loma, there is abnormal propagation of monoclonal immuno-
strated mild elevation of the medial left eyelid due to the mass globulins and/or light chains in the bone marrow, which
molding itself to the junction of the left medial canthal tendon and results in the diagnostic finding of monoclonal hypergamma-
scleral surface. Underlying scleral and medial canthal structures, globulinemia. The International Myeloma Working Group
including the anterior attachment of the medial rectus, appeared criteria require at least 10% plasma cells in the bone marrow
normal and otherwise preserved. or biopsy of a plasmacytoma and the presence of end organ
A unilateral epibulbar subconjunctival neoplasm represent- damage for the diagnosis of multiple myeloma.2
ing an ocular plasmacytoma was suspected. Excisional biopsy of
the lesion was performed. Histopathologic analysis was consistent
Ocular manifestations associated with multiple mye-
with a plasma cell neoplasm. Immunohistochemical stains demon- loma are separated into 2 groups; those secondary to
strated plasma cells with positive staining for CD138 and lambda- hematologic abnormalities associated with plasma cell
immunohistochemistry in the majority of plasma cells (Fig. 3). dyscrasia and those that are the direct result of tumor
Kappa-immunohistochemistry was negative in the majority of growth in ocular or surrounding structures. Because of this
plasma cells. In situ hybridization was negative for Epstein–Barr pathophysiology, ophthalmic involvement may occur in
virus and HHV8 in neoplastic cells. There was strong diffuse IgD virtually any ocular structure. Review of the literature
staining of the neoplastic cells, which correlated with the patient’s describes tumor affecting the orbit, conjunctiva, uvea,
known plasma cell multiple myeloma IgD variant. retina, lacrimal sac, and ciliary body.3–9 However, the
occurrence of an ocular plasmacytoma is rare and those
with conjunctival involvement are challenging to diag-
nose. Reported cases describe the appearance of lesions
with variable gross examinations including diffuse con-
junctival thickening and those which present as a well-
defined mass.7 Our patient presented with subconjunctival
hemorrhage likely obscuring the underlying growth.
Because of the inconsistent appearance of lesions, exam-
ination skills will often not suffice for diagnosis. To
differentiate a true plasmacytoma from an inflammatory
reaction, biopsy with histopathologic examination is
required for definitive diagnosis.
This case emphasizes the importance of maintaining
a high index of suspicion in patients with subconjunctival
hemorrhage with known or suspected multiple myeloma,
especially if the hemorrhage is recurrent. This patient with
known multiple myeloma presented with new-onset subcon-
junctival hemorrhage 4 months before observation of tumor
growth. We suspect that the subconjunctival hemorrhage
masked the presence of the plasmacytoma. The presence of
ophthalmic manifestations may be a sign of uncontrolled or
recurrent multiple myeloma. To our knowledge, there have
been no previous reports of subconjunctival hemorrhage
FIGURE 2. MRI demonstration of the left medial canthal delaying detection of subconjunctival plasmacytoma as
episcleral soft tissue mass. described in our patient.

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Cornea  Volume 36, Number 2, February 2017 Epibulbar Plasmacytoma in Patients With Myeloma

FIGURE 3. Immunohistochemical
stains of plasma cells: positive stain-
ing for CD138 (A) and lambda-
immunohistochemistry (B) in the
majority of plasma cells.

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