Baylor University Medical Center Proceedings October 2017 - 447

Natural killer/T-cell lymphoma invading the orbit and globe
Lance J. Lyons, MD, Ivan Vrcek, MD, Marie Somogyi, MD, Kevin Taheri, MD, Joan H. Admirand, MD,
Saradha Chexal, MD, Demetrius F. Loukas, MD, and Tanuj Nakra, MD

Natural killer/T-cell lymphomas are extremely rare and carry high mortality rates. Epidemiologically, these cancers tend to affect mainly Asian
and South American patients and are associated with Epstein-Barr virus
seropositivity. This report details a 78-year-old Vietnamese woman who
presented initially with vitritis of unknown cause, but later developed
proptosis and conjunctival involvement as her disease spread. Biopsies
of the orbit, ethmoid sinus, and conjunctiva were found to be significant
for natural killer/T-cell lymphoma. The case highlights the diagnostic
difficulty of this tumor given its rarity and ability to mimic other disorders.

ost lymphomas involving the orbit and globe are nonHodgkin's B-cell lymphomas (1). Only about 2% of
intraocular lymphomas are non-B-cell tumors, and
those expressing natural killer (NK) cell markers are
typically highly aggressive and carry a poor prognosis (2). Since
they arise primarily from the nasal mucosa, NK/T-cell lymphomas have the ability to invade the orbit and globe (2). Herein
we present a case of NK/T-cell lymphoma in a 78-year-old
Vietnamese woman who initially presented with vision problems and was successfully treated with chemotherapy.
CASE REPORT
A 78-year-old Vietnamese woman with a past medical history of coronary artery disease, hyperlipidemia, and hypothyroidism presented with a 6-month history of blurred vision in
the left eye associated with sporadic pressure-like pain in that
eye. On exam, vision in the left eye was 20/50, and there was
no improvement with pinhole testing. Her intraocular pressure
in the left eye was 10 mm Hg. The patient demonstrated mild
vitritis and periphlebitis with vascular sheathing in the temporal periphery on the left. She also manifested a pigmented
chorioretinal scar in the superonasal periphery of the same eye.
The remainder of the exam, including pupils and extraocular
movements, was normal.
The patient was initially diagnosed with uveitis and treated
with a dexamethasone intravitreal implant; however, she developed recurrence of symptoms after implant dissolution. Serologic workup was performed that included tests for antineutrophil
cytoplasmic antibody, toxoplasma IgM and IgG, rapid plasma
reagin, QuantiFERON tuberculosis, and Lyme antibodies, all

Proc (Bayl Univ Med Cent) 2017;30(4):447-449

of which were negative. The patient also demonstrated a normal erythrocyte sedimentation rate, C-reactive protein level,
and angiotensin-converting enzyme level. Magnetic resonance
imaging with contrast demonstrated a metallic foreign body
in the preseptal upper lid but was otherwise unremarkable and
notably negative for any mass lesions, including central nervous
system lymphoma, and an anterior chamber paracentesis failed
to yield diagnostic results.
Two months later, the patient returned with an inﬂamed
salmon-colored lesion within the medial conjunctiva of the left
eye and markedly decreased visual acuity on the left to 20/200.
Examination demonstrated supra-, infra-, and abduction deﬁcits
on the left with mechanical ptosis, as well as 5 mm of proptosis
and mild resistance to retropulsion.
Due to the presence of a metallic foreign body, a contrastenhanced computed tomography (CT) scan of the head was
substituted for magnetic resonance imaging. This scan demonstrated extensive opaciﬁcation of the left maxillary and ethmoid
sinuses, as well as an invasive pattern of hyperdense material
ﬁlling the majority of the left intraconal space, which extended
anteriorly, causing protrusion of the subcutaneous tissue of the
inferior eyelid (Figure 1).
The patient underwent orbitotomy with biopsy of the abnormal tissue in the left orbit and ethmoid sinus as well as
a conjunctival biopsy. Pathology results revealed intermediate- to large-sized neoplastic CD2+, CD56+, CD5+ lymphoid
cells, highlighting an atypical prominent population of true
NK cells (Figure 2). In situ hybridization for Epstein-Barr virus
was strongly and diﬀusely positive in neoplastic cells. The diagnosis of extranodal NK/T-cell lymphoma nasal type was made,
and the patient underwent tumor staging. A positron emission
tomography (PET) scan demonstrated extensive metabolically
active disease, indicating a stage IVA diagnosis. The patient
received three of six planned cycles of gemcitabine, oxaliplatin,
From the University of Texas at Austin Dell Medical School, Austin, Texas (Lyons);
TOC Eye and Face, Austin, Texas (Vrcek, Somogyi, Nakra); ENT and Allergy Center
of Austin, Round Rock, TX (Taheri); Seton Medical Center Austin, Austin, Texas
(Admirand); Retina Consultants of Austin, Austin, Texas (Chexal); and Texas
Oncology, Austin, Texas (Loukas).
Corresponding author: Ivan Vrcek, MD, TOC Eye and Face, 3705 Medical
Parkway, Suite 120, Austin, TX 78705 (e-mail: ivan.vrcek@gmail.com).
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