James English, Timothy Sullivan
To characterise the clinical features, inves-tigations, treatments, complications and outcomes ofrare T-cell origin Ocular Adnexal Lymphomas.
Retrospective case series over 20 years.Chart reviews of 10 cases were conducted.
Ten patients were selected, 5 male, 5 femalewith mean age of 44 (range 7–75). Presenting symp-toms ranged from a painless conjunctival mass toblurred vision, headaches and V2 distribution anaes-thesia. Three cases involved primary disease with sevenin the setting of systemic disease. Two patients hadNK/T-Cell lymphoma, 5 patients had Anaplastic Large TCell lymphoma, two had cutaneous T cell lymphomaand one had a Peripheral T Cell lymphoma. Treatmentinvolved radiotherapy in 5 cases and chemotherapy in 6cases. Overall survival was 59 months (range 2–124)with mean freedom from systemic relapse of 45 months(range 18–124). Two patients suffered extra orbital relapses. There were no local recurrences for non-progressive disease. Both patients with NK/T-Cell lym-phoma died within three months. Four patients in totalsuccumbed to disease or complications of treatment,one died from other causes and ?ve remain disease free.
This study shows T-Cell ocular adnexallymphomas can affect all ages, present in numerousways and is often a manifestation of systemic diseasewith imminent threat to life. Distant Relapses occurwarranting long term surveillance. A diagnosis of T-celllymphoma should be entertained in non-resolvingperi-ocular skin lesions. This study con?rms theaggressive, relentless progression of T/NK cell neo-plasms of the ocular adnexa and highlights the impor-tance of a multidisciplinary approach.