Baylor University Medical Center Proceedings October 2017 - 448

NK/T-cell lymphoma does occasionally invade the globe or ocular
adnexa and represents approximately 1% to 3% of all lymphoproliferative lesions in these locations (4).
Over 70% of these cancers localize
to the nasopharynx with inflammation and local tissue destruction
(5); however, angioinvasion allows
widespread dissemination to the
gastrointestinal tract (6), skin (7),
and central nervous system (8). A
recent review that included 24 patients with primary nasal or nasopharyngeal NK/T-cell lymphoma
identified six patients (25% of the
study population) who suffered
Figure 1. A CT scan taken after the development of left-sided proptosis shows (a) hyperdense material filling the left vision-threatening complications
intraconal space and protruding into the inferior eyelid and (b) prominent maxillary and ethmoid sinus opacification.
that stemmed from uveitis/vitritis
and/or orbital inflammation (5). Intraocular and orbital presand L-asparaginase (GELOX) chemotherapy. Her therapy was
entations of the malignancy have been confused with a range
suspended due to severe treatment-induced debilitation; however, she remains in complete remission by both CT and PET
of diagnoses, including orbital cellulitis (9, 10), nonspecific
scan as of her most recent follow-up 10 months following her
orbital inflammation (6), conjunctivitis, anterior uveitis, and
initial oncologic consultation.
optic neuritis (11).
The prognosis of extranodal NK/T-cell lymphomas is worse
DISCUSSION
than that of their B-cell counterparts (12). While early stage,
NK/T-cell lymphoma is a rare and aggressive subtype most
localized disease is highly curable, metastatic disease and refractory cases carry a 5-year survival rate of <10% (13). Treatcommon in Asian and South American populations and asment for localized disease (stage I/II) includes chemotherapy
sociated with EBV (3). While most ocular and orbital lymand radiotherapy; however, disseminated disease is treated with
phomas are of the non-Hodgkin's B-cell variety, the nasal type
b

a

a

b

d

c

e

Figure 2. Pathology results confirming the diagnosis of natural killer/T-cell lymphoma: (a) in situ hybridization for Epstein Barr virus on orbital tissue showing intermediate- to large-sized neoplastic cells with nuclear irregularities that stain strongly positive (1000×); (b) CD3 immunohistochemical stain of orbital tissue (1000×);
(c) CD5 immunohistochemical stain of orbital tissue (1000×); (d) CD56 immunohistochemical stain of orbital tissue (1000×); (e) sinonasal mucosa with an abnormal
lymphoid infiltrate displaying some nuclear irregularity underlying normal respiratory epithelium (200×).
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