Clinical characteristics and treatment outcomes of natural killer/T-cell lymphoma involving the ocular adnexa.
AIMS
To evaluate the clinical characteristics and treatment outcomes of natural killer/T-cell lymphoma (NKTL) involving the ocular adnexa.


METHODS
Retrospective, comparative, observational case series. A total of 350 patients with NKTL, including 27 patients with NKTL involving the ocular adnexa from 1999 to 2016. The patients were grouped into two groups: group 1 comprised patients presenting with ophthalmic symptoms, and group 2 comprised patients presenting with symptoms from other organs but subsequently developed ophthalmic involvement.


RESULTS
Group 1 comprised 12 patients (44.4%) and group 2 comprised 15 (55.6%). Mean duration of symptoms in group 1 was 1.8±1.2 months, while the time from diagnosis of NKTL to development of ophthalmic symptoms in group 2 was 45.3±65.6 months. Periorbital swelling was the most common presenting symptom in both groups (83.3% in group 1 and 73.3% in group 2). Symptoms mimicking cellulitis and pseudotumor were present in 50.0% and 16.7% of cases, respectively. The 5-year overall survival rate was 18.5% in group 1 and 26.4% in group 2, while the 5-year progression-free survival rate was 0% and 13.3%, respectively.


CONCLUSIONS
Our series is to our knowledge the largest cohort study on NKTL reported to date and demonstrates that ocular adnexal NKTL is a rare but seriously fatal disease. It is characterised by acute inflammatory signs as present in as many as two-thirds of our patients in this series. It should be considered as a differential diagnosis in patients presenting with rapidly progressing proptosis and diagnosed promptly for optimal management.
['Lee|Ga-In|GI|', 'Kim|Yoon-Duck|YD|', 'Young|Stephanie Ming|SM|', 'Shin|Seonae|S|', 'Woo|Kyung In|KI|']
D000293:Adolescent D000328:Adult D000368:Aged D000971:Antineoplastic Combined Chemotherapy Protocols D015331:Cohort Studies D018572:Disease-Free Survival D005260:Female D006801:Humans D007694:Killer Cells, Natural D016399:Lymphoma, T-Cell D008297:Male D008444:Maxillary Sinus Neoplasms D008875:Middle Aged D009062:Mouth Neoplasms D009669:Nose Neoplasms D009918:Orbital Neoplasms D012189:Retrospective Studies D015996:Survival Rate D016896:Treatment Outcome D055815:Young Adult
masquerading syndrome natural killer T-cell lymphoma ocular adnexal lymphoma survival analysis