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Case report and literature review of ENKTL presenting with ocular symptoms and treated with GemOxA Rare Eye Symptom Led Doctors Down the Wrong Path for Months

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Key Takeaway
Consider ENKTL in differential when orbital symptoms mimic inflammation despite imaging.

This publication is a case report with literature review focusing on extranodal natural killer/T cell lymphoma (ENKTL) presenting with sinusitis and orbital cellulitis symptoms. The authors describe a single patient who received gemcitabine/oxaliplatin (GemOx) chemotherapy after functional endoscopic sinus surgery and a second surgical procedure. After five rounds of chemotherapy, the patient remained in remission with no evidence of lymphoma recurrence, though vision loss in the right eye occurred as an adverse event.

The authors note that diagnosis is particularly challenging when patients present with facial swelling and periocular edema as initial symptoms, especially when multiple CT and MRI examinations suggest inflammatory lesions rather than malignancy. They suggest blindness may be caused by compression of the optic nerve due to swelling of orbital tissues, though this is based on a single case observation.

Significant limitations include that this is a rare case report of only 1 patient, with no comparator group or statistical analysis. The authors acknowledge the diagnostic challenges when ENKTL presents with ocular symptoms that mimic inflammatory conditions. As a case report, these findings represent anecdotal evidence that cannot establish treatment efficacy or safety profiles.

For clinical practice, this case highlights the diagnostic difficulty when ENKTL presents with orbital symptoms that resemble inflammatory conditions. The reported remission with GemOx chemotherapy after surgery in this single patient should be viewed as preliminary observation only. Clinicians should recognize this as a rare presentation requiring careful differential diagnosis.

Double Vision, Wrong Diagnosis

He walked into the clinic with double vision. His sinuses looked clear. His scans looked clean. His doctors treated him for a sinus infection and sent him home.

Weeks later, he was back — and losing his sight.

A Lymphoma That Hides in Plain Sight

Extranodal NK/T cell lymphoma, or ENKTL, is a rare type of non-Hodgkin lymphoma (a cancer of the lymph system that starts outside the lymph nodes). Unlike most blood cancers, ENKTL tends to grow in soft tissues of the face and upper airway — the nose, sinuses, and palate. It is strongly linked to the Epstein-Barr virus (EBV), the same virus that causes mono.

What makes ENKTL especially dangerous is how well it can disguise itself. Early in the disease, the tissue changes are subtle. Standard imaging — CT scans and MRIs — can look almost normal. And the symptoms? Facial swelling, nasal congestion, puffy eyes. The exact same things you'd see with a bad sinus infection.

When Scans Say One Thing and the Body Says Another

In this case, the patient's first nasal endoscopy (a camera inspection inside the nose) looked normal. His first MRI and CT showed no obvious mass. He was diagnosed with sinusitis and had surgery to open his sinuses — a common procedure called FESS (functional endoscopic sinus surgery).

But his condition worsened after surgery. A follow-up CT scan showed subtle changes near the muscle that controls downward eye movement. A suspicious area appeared near a gap in the bone at the base of the eye socket. Doctors now suspected orbital cellulitis — a bacterial infection of the tissue around the eye.

This doesn't mean ENKTL is always this hard to find, but when it mimics infection, multiple rounds of testing may be needed before the truth emerges.

The first tissue biopsy (removing a small piece of tissue for analysis) did not show cancer. It took a second biopsy. Then a third — samples taken from six different sites — combined with a test for Epstein-Barr virus, to finally confirm ENKTL.

How Tissue Testing Finally Cracked the Case

Think of cancer diagnosis like reading a hidden message — each biopsy gives you more letters. One sample from one location may not spell anything clear. But six samples from six sites, read alongside a positive EBV test, can finally reveal the word.

The EBV connection is key. ENKTL cells almost always carry traces of this virus, and detecting EBV in tissue is one of the most reliable ways to confirm the diagnosis when the standard cancer markers are ambiguous.

What They Found — and the Cost of Delay

By the time the diagnosis was confirmed, the patient had already lost vision in his right eye. Swelling from the growing lymphoma had pressed on the optic nerve long enough to cause permanent damage. Emergency surgery was performed to try to save his vision, but the window had already closed.

He then received chemotherapy — specifically a combination called GemOx (gemcitabine and oxaliplatin). After five rounds, his lymphoma went into remission (the cancer is no longer detectable). He remains in remission with no signs of recurrence.

That's Not the Full Story

The most important finding here is not the treatment — chemotherapy for ENKTL is not new. The real lesson is timing. Every week of misdiagnosis gave the tumor more time to press on the optic nerve. By the time the cancer was named, an irreversible outcome had already occurred.

Clinicians reviewing this case note that when facial swelling, eye symptoms, or tissue around the eye fails to respond to standard infection treatment, ENKTL should be on the radar — even if early scans look reassuring.

If you or someone you know has persistent swelling around the eyes, double vision, or facial puffiness that does not get better with antibiotics or sinus treatment, push for a specialist referral. Ask whether a tissue biopsy has been done — and if so, whether it was sent for both standard pathology and viral testing (specifically EBV).

This is rare, but it is not vanishingly rare. And it is treatable when caught early.

This is a single case report from one hospital. Case reports describe one patient's experience and cannot tell us how common a pattern is, or whether the same sequence would play out differently elsewhere. ENKTL is more prevalent in East Asia and Latin America than in Western countries, and the presentation described here — with purely eye-based symptoms at the start — is considered unusual even for this cancer.

This case adds to a small but growing body of literature urging clinicians to keep ENKTL on the differential (the list of possible diagnoses) whenever patients present with eye and facial symptoms that resist standard infection treatment. Researchers are working to identify biomarkers (measurable signals in blood or tissue) that could flag ENKTL earlier, before imaging and biopsies produce clear results. Faster, less invasive diagnostic tools could one day compress a months-long diagnostic journey into days.

Study Details

Study typeSystematic review
EvidenceLevel 1
PublishedApr 2026
View Original Abstract ↓
Extranodal natural killer/T cell lymphoma (ENKTL) is a non-Hodgkin lymphoma (NHL) with extranodal presentation. This report presents a rare case of ENKTL with ocular symptoms as the initial manifestation. Nasal endoscopy was normal. Magnetic resonance imaging (MRI) and sinus computed tomography (CT) scan revealed no evidence of mass or lymphadenopathy. The first hospitalization was misdiagnosed as sinusitis. Ophthalmic examination showed no significant decrease in vision. On 15 April 2025, a functional endoscopic sinus surgery (FESS) was performed. However, the patient’s condition worsened, leading to a second hospitalization. Postoperative CT shows changes in the area of the right inferior rectus muscle below the eyeball compared with preoperative CT, and the initial CT also showed a suspicious space-occupying lesion in the right orbital inferior orbital fissure region. Imaging examinations suggested orbital cellulitis. The first histopathological examination of the local mucosa did not provide a definitive tumor diagnosis. The second pathological examination was conducted. The patient lost vision in the right eye before the second set of pathological results came out. Based on examination results, the blindness may be caused by compression of the optic nerve due to swelling of the orbital tissues. A second surgery was performed urgently to restore the patient’s vision as soon as possible. Samples from six sites were sent for a third histopathological examination, and combined with the detection of Epstein–Barr virus (EBV), ENKTL was revealed, as in the second examination. The patient received gemcitabine/oxaliplatin (GemOx) chemotherapy. After five rounds of chemotherapy, he remains in remission, with no evidence that the lymphoma has recurred. It is relatively rare for ENKTL to involve intraocular or ocular adnexal tissues. The diagnosis is particularly challenging when patients present with facial swelling and periocular edema as the initial symptoms, especially when multiple CT and MRI examinations suggest the possibility of inflammatory lesions. When visiting our hospital, this patient presented with diplopia as the initial ophthalmologic complaint and ended up losing vision in the right eye, which was another “take-away” lesson of this case.
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