A case of extranodal NK/T-cell lymphoma presenting visual loss

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We report the clinical course of a 74 year-old man with extranodal NK/T cell lymphoma, nasal type (ENKL). He developed gradual right visual loss for several months. At the referral to our clinic, he presented swelling of the eyelid, but endoscopic observation of nasal cavity revealed no signs of nasal cavity tumor. Together with computed tomographic scan, we initially assumed his case as invasive sinus aspergillosis, that we immediately performed endoscopic sinus surgery. The paranasal sinus biopsy in the surgery showed diffuse infiltrates of lymphoma cells and inflammatory cells accompanied with wide spread ischemic necrosis, and he was diagnosed of ENKL. Finally, with positive cytology of cerebrospinal fluid and aqueous humor from both eyes, this case was classified into Ann Arbor IV B stage. Radiation therapy was performed and primary lesion was controlled, but his general condition gradually deteriorated, and he died in about three months after diagnosis. ENKL, an uncommon lymphoproliferative disorder, usually involve the nasal cavity or paranasal sinuses, and rarely occurs in the orbit. But in this case, we suppose the origin of ENKL to be the orbit. Early diagnosis of ENKL is important, because ENKL of advanced clinical stages shows poor prognosis. To make early diagnosis, biopsy of several tissues and immunostaining are desirable because of necrotic tissue contained in tumor. It is also important to inform pathologist about possibility of ENKL for immunostaining.

A case of extranodal NK/T-cell lymphoma presenting visual loss

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