Extranodal nasal-type NK/T lymphoma treated with chemotherapy and radiotherapy: case series from a European tertiary referral center and review of the literature

Purpose Extranodal nasal-type NK/T-cell lymphoma (ENKTL) is very rare in western countries and few data are available regarding the prognosis and the outcome of patients treated for this disease. We aimed to evaluate the prognosis, the pattern and risk factors of disease failure after combined therapy and also performed a review of the literature. Patients and methods We retrospectively analyzed 20 patients with (ENKTL) who underwent L‑Asparaginase based chemotherapy followed by (chemo-) radiotherapy between 2010 and 2020 in our center. Data on clinical characteristics and irradiation were collected. Failure patterns were recorded as local (tumor site), regional (regional lymph nodes) or distant failure (metastasis and/or nonregional lymph nodes). Results During a median follow-up period of 46 months, disease failure was observed in 8 patients (40%). The 3‑year progression-free survival (PFS) and overall survival (OS) rates were 62.5 and 83.0%, respectively. The failure patterns were local ( n = 6, 30%), regional ( n = 3, 15%) and distant ( n = 4, 20%). Among patients with local failure, all failures occurred within the radiation fields (100%). Univariate analysis showed that bilateral regional lymph node involvement ( p = 0.0002), initial circulating EBV viral load ≥ 3.5 log ( p = 0.03) and no negativation of EBV PCR after induction CT ( p = 0.0497) were independent predictors of PFS. Conclusion Patients with bilateral lymph node involvement and/or high EBV viral load have a significant recurrence rate despite multimodal therapy. These results need to be confirmed by larger studies. Given the high rate of local recurrence within radiotherapy fields, the value of dose escalation should be considered. Patients at risk of relapse should be included in dedicated trials.