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Treatment and survival for patients with localized primary ocular adnexal extranodal marginal zone lymphoma

Leukemia volume 38, pages 914–917 (2024)Cite this article

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Primary ocular adnexal extranodal marginal zone lymphoma (OA-EMZL) represents the second most common EMZL [1,2,3] and the predominant type of ocular adnexal lymphoma [4], with a favorable prognosis and an excellent 5-year overall survival (OS) rate ( > 95%) [3]. Guidelines recommend radical radiotherapy (RT) as the standard of care for localized OA-EMZL [5, 6]. In real-world practice, early-stage lesions are diversely managed with RT, observation, chemotherapy, immunotherapy, and/or surgical resection, depending on the age and performance status (PS) of the patient, the disease extent, and other clinical factors [4, 7]. In the past two decades, a significant trend toward omission of RT for early-stage EMZL has been recorded [2, 8], probably because of concerns regarding long-term toxicity [2]. This trend has been particularly prominent in China, and data regarding EMZL are notably scarce. However, analysis of the association between treatment and survival in a large dataset showed results favoring primary RT over no RT, with a >10% difference in both OS and relative survival (RS) [2]. Moreover, data on long-term toxicities and quality of life (QoL) after different treatments are limited [9,10,11]. More data regarding progression, survival, and impact on QoL based on specific sites and long-term follow-up are needed to optimize the management of EMZL.

In this study, data for patients with histologically diagnosed, stage IE OA-EMZL between January 2000 and December 2020 were retrospectively reviewed at Cancer Hospital of Chinese Academy of Medical Sciences (a specialized cancer hospital) and Beijing TongRen Hospital (a comprehensive hospital specializing in ophthalmology). The Supplementary Methods show the inclusion criteria. In total, 262 patients were recruited. The baseline characteristics are summarized in Table S1, while the previous medical histories and anatomical locations are summarized in Table S2. Briefly, the median age was 55 years, 60 patients (22.9%) had bilateral lesions, and 84 patients (32.1%) showed tumors at multiple sites within the ocular adnexa.

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Fig. 1: Survival and mortality for all patients.
Fig. 2: Cumulative failure rates according to the first-line treatment.

Data availability

The data that support the findings of this study are available from the corresponding author upon reasonable request.

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Funding

This work was supported by grants from the Beijing Hope Run Special Fund of Cancer Foundation of China [grant number LC2021A01]; the National Natural Science Foundation of China [grant number 82170181]; the Beijing Natural Science Foundation [grant number 7222027]; the National Key R&D Program of China [grant number 2022YFF1502000]; and the Fundamental Research Funds for the Central Universities [grant number 3332022028].

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Author notes

  1. These authors contributed equally: Lin-Rui Gao, Xin Li, Xinyue Wang.

Authors and Affiliations

  1. Department of Radiation Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences (CAMS) and Peking Union Medical College (PUMC), Beijing, 100021, China

    Lin-Rui Gao, Xinyue Wang, Yunpeng Wu, Xin Liu, Yong-Wen Song, Hui Fang, Bo Chen, Jing Jin, Yue-Ping Liu, Hao Jing, Yuan Tang, Ning-Ning Lu, Shu-Lian Wang, Ye-Xiong Li & Shu-Nan Qi

  2. Department of Hematology, Beijing TongRen Hospital, Capital Medical University, Beijing, 100730, China

    Xin Li, Yuanzheng Liang & Liang Wang

  3. Department of Pathology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences (CAMS) and Peking Union Medical College (PUMC), Beijing, 100021, China

    Xiao-Li Feng & Wei Rao

Authors
  1. Lin-Rui Gao
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Contributions

SQ, LW, YL, LG, and XL designed the study; LG and XL analyzed the data and made the figures; SQ, LW, YL, LG, XL, and XW wrote the manuscript; SQ and LW contributed to the study coordination; All authors contributed to data collection and interpretation, and finally approved the manuscript. Part of this work was accepted for Proffered oral presentation at the European Society for Medical Oncology (ESMO) Congress 2023, Madrid, Spain, 20–24 October 2023.

Corresponding authors

Correspondence to Ye-Xiong Li, Liang Wang or Shu-Nan Qi.

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Gao, LR., Li, X., Wang, X. et al. Treatment and survival for patients with localized primary ocular adnexal extranodal marginal zone lymphoma. Leukemia 38, 914–917 (2024). https://doi.org/10.1038/s41375-024-02227-5

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