Knudsen MKH, et al. - This retrospective multicenter study entailed characterization of the clinical features of ocular adnexal mantle-cell lymphoma (OA-MCL). Appearance in older male individuals, advanced stage and bilateral manifestation at the time of diagnosis, and aggressive course were identified as the distinctive features of OA-MCL. In addition, rituximab plus chemotherapy might improve prognosis.

Methods

• In this retrospective multicenter study, data collected from January 1, 1980, through December 31, 2015, at 6 eye cancer centers in 4 countries, was included.
• Researchers reviewed medical records of 55 patients with OA-MCL; the median length of follow-up was 33 months.
• The primary end points were overall survival, disease-specific survival, and progression-free survival.

Results

• This analysis included a total of 55 patients.
• Data showed that ocular adnexal MCL was most common in older individuals (mean age, 70 years) and men (n = 42 of 55; 76%).
• A frequent presentation with disseminated lymphoma (n = 34 of 55; 62%) was seen in patients with OA-MCL, and these patients were likely to experience stage IVE disease (n = 35 of 55; 64%), with bilateral involvement (n = 27 of 55; 47%), tumor masses (n = 27 of 36; 75%), and involvement of the orbit (n = 32 of 55; 58%).
• Researchers found that the most frequently used treatment was chemotherapy with or without external beam radiation therapy.
• Results revealed that overall survival rates for the entire cohort were 65% at 3 years (95% CI, 52%-78%) and 34% at 5 years (95% CI, 21%-47%).
• After 5 years, the reported disease-specific survival was 38% for the entire cohort (95% CI, 25%-51%); in patients who had received rituximab in addition to the chemotherapy regimen, the disease-specific survival adjusted by eye cancer center was better (hazard ratio, 3.3; 95% CI, 1.0-14.7; P=.06).
• Researchers noted that in patients who experienced recurrence after primary treatment and in patients who presented with a relapse of systemic lymphoma in the ocular adnexal region, the median progression-free survival was 2.3 years (95% CI, 1.8-2.7 years) and 4.1 years (95% CI, 3.9-4.3 years), respectively.