a 57-year - old woman presented with a three - day history of photophobia and diplopia in the left eye .
she was diagnosed with histologically confirmed nasal nktl of the right nasal cavity one month prior .
the snellen visual acuity of the affected eye with correction was 1.0 / 0.63 , and the intraocular pressure in the right and left eyes was 17 and 18 mmhg , respectively .
the patient had mild left eye conjunctival injection , ptosis , and swelling of the eyelid .
opthalmoplegia , vitreous opacity , and an iris mass developed in the left eye ( fig .
thirty - five percent of the cells obtained from the aspiration were morphologically atypical lymphocytes .
skin biopsy confirmed neoplastic infiltration of small- to medium - sized pleomorphic lymphocytes with irregular nuclei , inconspicuous nucleoli , and scant cytoplasm ( fig .
immunohistochemical staining was positive for cd56 ( fig . 2c ) and cd3 ( fig .
the patient was diagnosed with nasal nktl with ocular involvement . after receiving radiotherapy at 900 cgy ,
her visual acuity improved and her anterior uveitis , vitreous opacity , and iris mass resolved ( fig .
3a and 3b ) . however , the patient 's condition continued to deteriorate despite radiotherapy and chemotherapy , and her ptosis and ophthalmoplegia gradually progressed .
extranodal nktl , including nasal nktl , previously known as lethal midline granuloma , is a definitive diagnostic entity according to the world health organization lymphoma classification .
however , histopathologically identical tumors may be identified at other extranodal sites , including the skin , muscle , gastrointestinal tract , liver , kidney , and retroperitoneal space .
did report a case of histologically confirmed nasal nktl with intraocular involvement , however , in most cases intraocular involvement has not been confirmed histologically .
the presence of intraocular involvement raises the possibility of leptomeningeal or central nervous system dissemination , and prompts consideration for external - beam radiotherapy to the eye and orbit in addition to systemic chemotherapy .
nktl involving the ocular adnexa is generally a rapidly progressing disease , with a short survival from time of diagnosis , despite standard therapy .
this was true in the current case as well ; the patient died three months after diagnosis .
since nktl with ocular and orbital involvement is very rare and is characterized by rapid disease progression and a poor prognosis , nktl should be considered in the differential diagnosis of uveitis or orbital pseudotumor refractory to therapy .
 herein , we report a case of nasal natural killer t - cell lymphoma ( nktl ) with intraocular involvement . a 57-year - old woman was referred due to a three - day history of photophobia and diplopia in the left eye . 
 one - month previously , she was diagnosed with nasal nktl of the right nasal cavity . 
 ophthalmic examination revealed conjunctival injection and ptosis . 
 the left pupil was fully dilated and non - reactive to light . 
 ocular motion was restricted on left - upper gaze . 
 five days later , anterior uveitis developed and persisted despite topical steroid treatment . 
 an orbital magnetic resonance imaging was without specific findings , however , ophthalmoplegia , vitreous opacity , and an iris mass were observed . 
 a diagnostic anterior chamber aspiration was performed . 
 aqueous humor aspiration revealed 35% morphologically atypical lymphocytes . 
 after an intravitreal triamcinolone injection , radiotherapy and chemotherapy were administered ; this resolved the uveitis and iris mass . 
 when refractory uveitis or orbital pseudotumor occurs in patients with nasal nktl , ocular and orbital involvement of the nktl should be considered . 
Case Report
Discussion