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

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Abstract

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.

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Authors and Affiliations

  1. Radiation Oncology department, AP-HP, Hopital Saint Louis, 1, Avenue Claude Vellefaux, 75010, Paris, France

    Judith Porte, Christophe Hennequin, Sophie Guillerm &  Laurent Quéro MD PhD

  2. UMR/CEA, Immuno-Hematology Research Unit (SRHI), Université Paris Cité, Paris, France

    Christophe Hennequin

  3. Hematology department, AP-HP, Hopital Saint Louis, Paris, France

    Daphné Krizch & Catherine Thieblemont

  4. Nuclear Medicine Department, Hopital Saint Louis AP-HP, Paris, France

    Laetitia Vercellino

  5. INSERM, UMR_S942 MASCOT, Université Paris Cité, Paris, France

    Laetitia Vercellino

  6. INSERM U728, Universite Paris Cite, Paris, France

    Catherine Thieblemont

  7. INSERM U1160, Universite Paris Cite, Paris, France

    Laurent Quéro MD PhD

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  1. Judith Porte
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Correspondence to Laurent Quéro MD PhD.

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Conflict of interest

J. Porte, C. Hennequin, D. Krizch, L. Vercellino, S. Guillerm, C. Thieblemont and L. Quéro declare that they have no competing interests.

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For this article no studies with human participants or animals were performed by any of the authors. All studies mentioned were in accordance with the ethical standards indicated in each case.

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Porte, J., Hennequin, C., Krizch, D. et al. Extranodal nasal-type NK/T lymphoma treated with chemotherapy and radiotherapy: case series from a European tertiary referral center and review of the literature. Strahlenther Onkol 200, 434–443 (2024). https://doi.org/10.1007/s00066-023-02165-8

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