Review

. 2022 Sep 29;15(1):136.

doi: 10.1186/s13045-022-01356-7.

Evidence-based expert consensus on the management of primary central nervous system lymphoma in China

Tong Chen¹, Yuanbo Liu², Yang Wang^{3

4}, Qing Chang⁵, Jinsong Wu^{6

7}, Zhiliang Wang⁸, Daoying Geng⁹, Jin-Tai Yu¹⁰, Yuan Li⁹, Xiao-Qiu Li¹¹, Hong Chen¹², Dongxiao Zhuang⁶, Jianyong Li¹³, Bin Wang¹⁴, Tao Jiang¹⁵, Lanting Lyu¹⁶, Yuqin Song¹⁷, Xiaoguang Qiu¹⁸, Wenbin Li¹⁹, Song Lin¹⁵, Xinghu Zhang²⁰, Dehong Lu²¹, Junqiang Lei²², Yaolong Chen^{23

24

25}, Ying Mao^{26

27}

Affiliations

¹ Department of Hematology, Institute of Medicine, Huashan Hospital, Fudan University, Shanghai, 200040, China. chentong@fudan.edu.cn.
² Department of Hematology, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100070, China.
³ Department of Radiation Oncology, Huashan Hospital, Fudan University, Shanghai, 201107, China.
⁴ National Center for Neurological Disorders, Huashan Hospital, Fudan University, Shanghai, 200040, China.
⁵ Department of Ophthalmology, Eye and ENT Hospital, Fudan University, Shanghai, 200031, China.
⁶ Department of Neurosurgery, National Center for Neurological Disorders, Huashan Hospital, Fudan University, Shanghai, 200040, China.
⁷ Institute of Medicine, Huashan Hospital, Fudan University, Shanghai, 200040, China.
⁸ Department of Ophthalmology, Huashan Hospital, Fudan University, Shanghai, 200040, China.
⁹ Department of Radiology, Huashan Hospital, Fudan University, Shanghai, 200040, China.
¹⁰ Department of Neurology, Huashan Hospital, Fudan University, Shanghai, 200040, China.
¹¹ Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai, 200032, China.
¹² Department of Pathology, Huashan Hospital, Fudan University, Shanghai, 200040, China.
¹³ Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing, 210029, China.
¹⁴ Department of Pharmacy, Huashan Hospital, Fudan University, Shanghai, 200040, China.
¹⁵ Department of Neurosurgery, Beijing Neurosurgical Institute, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100070, China.
¹⁶ School of Public Administration and Policy, Health Technology Assessment and Policy Evaluation Group, Renmin University of China, Beijing, 100872, China.
¹⁷ Department of Lymphoma, Peking University Cancer Hospital and Institute, Beijing, 100142, China.
¹⁸ Department of Radiation Oncology, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100070, China.
¹⁹ Department of Neuro-Oncolgoy, Cancer Center, National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100070, China.
²⁰ Department of Neurology, Neuroimmunology and Neuroinfection Center, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100070, China.
²¹ Department of Pathology, Xuanwu Hospital, Capital Medical University, Beijing, 100053, China.
²² Department of Radiology, The First Hospital of Lanzhou University, Lanzhou, 730000, China.
²³ Research Unit of Evidence-Based Evaluation and Guidelines, Chinese Academy of Medical Sciences (2021RU017), School of Basic Medical Sciences, Lanzhou University, Lanzhou, 730000, China. chenyaolong@lzu.edu.cn.
²⁴ WHO Collaborating Center for Guideline Implementation and Knowledge Translation, Lanzhou University, Lanzhou, 730000, China. chenyaolong@lzu.edu.cn.
²⁵ Lanzhou University GRADE Center, Lanzhou, 730000, China. chenyaolong@lzu.edu.cn.
²⁶ Department of Neurosurgery, National Center for Neurological Disorders, Huashan Hospital, Fudan University, Shanghai, 200040, China. maoying@fudan.edu.cn.
²⁷ Institute of Medicine, Huashan Hospital, Fudan University, Shanghai, 200040, China. maoying@fudan.edu.cn.

PMID: 36176002
PMCID: PMC9524012
DOI: 10.1186/s13045-022-01356-7

Review

Evidence-based expert consensus on the management of primary central nervous system lymphoma in China

Tong Chen et al. J Hematol Oncol. 2022.

. 2022 Sep 29;15(1):136.

doi: 10.1186/s13045-022-01356-7.

Authors

Affiliations

¹ Department of Hematology, Institute of Medicine, Huashan Hospital, Fudan University, Shanghai, 200040, China. chentong@fudan.edu.cn.
² Department of Hematology, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100070, China.
³ Department of Radiation Oncology, Huashan Hospital, Fudan University, Shanghai, 201107, China.
⁴ National Center for Neurological Disorders, Huashan Hospital, Fudan University, Shanghai, 200040, China.
⁵ Department of Ophthalmology, Eye and ENT Hospital, Fudan University, Shanghai, 200031, China.
⁶ Department of Neurosurgery, National Center for Neurological Disorders, Huashan Hospital, Fudan University, Shanghai, 200040, China.
⁷ Institute of Medicine, Huashan Hospital, Fudan University, Shanghai, 200040, China.
⁸ Department of Ophthalmology, Huashan Hospital, Fudan University, Shanghai, 200040, China.
⁹ Department of Radiology, Huashan Hospital, Fudan University, Shanghai, 200040, China.
¹⁰ Department of Neurology, Huashan Hospital, Fudan University, Shanghai, 200040, China.
¹¹ Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai, 200032, China.
¹² Department of Pathology, Huashan Hospital, Fudan University, Shanghai, 200040, China.
¹³ Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing, 210029, China.
¹⁴ Department of Pharmacy, Huashan Hospital, Fudan University, Shanghai, 200040, China.
¹⁵ Department of Neurosurgery, Beijing Neurosurgical Institute, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100070, China.
¹⁶ School of Public Administration and Policy, Health Technology Assessment and Policy Evaluation Group, Renmin University of China, Beijing, 100872, China.
¹⁷ Department of Lymphoma, Peking University Cancer Hospital and Institute, Beijing, 100142, China.
¹⁸ Department of Radiation Oncology, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100070, China.
¹⁹ Department of Neuro-Oncolgoy, Cancer Center, National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100070, China.
²⁰ Department of Neurology, Neuroimmunology and Neuroinfection Center, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100070, China.
²¹ Department of Pathology, Xuanwu Hospital, Capital Medical University, Beijing, 100053, China.
²² Department of Radiology, The First Hospital of Lanzhou University, Lanzhou, 730000, China.
²³ Research Unit of Evidence-Based Evaluation and Guidelines, Chinese Academy of Medical Sciences (2021RU017), School of Basic Medical Sciences, Lanzhou University, Lanzhou, 730000, China. chenyaolong@lzu.edu.cn.
²⁴ WHO Collaborating Center for Guideline Implementation and Knowledge Translation, Lanzhou University, Lanzhou, 730000, China. chenyaolong@lzu.edu.cn.
²⁵ Lanzhou University GRADE Center, Lanzhou, 730000, China. chenyaolong@lzu.edu.cn.
²⁶ Department of Neurosurgery, National Center for Neurological Disorders, Huashan Hospital, Fudan University, Shanghai, 200040, China. maoying@fudan.edu.cn.
²⁷ Institute of Medicine, Huashan Hospital, Fudan University, Shanghai, 200040, China. maoying@fudan.edu.cn.

PMID: 36176002
PMCID: PMC9524012
DOI: 10.1186/s13045-022-01356-7

Abstract

Primary central nervous system lymphoma (PCNSL) is a type of central nervous system restricted non-Hodgkin lymphoma, whose histopathological diagnosis is majorly large B cell lymphoma. To provide specific, evidence-based recommendations for medical professionals and to promote more standardized, effective and safe treatment for patients with PCNSL, a panel of experts from the Chinese Neurosurgical Society of the Chinese Medical Association and the Society of Hematological Malignancies of the Chinese Anti-Cancer Association jointly developed an evidence-based consensus. After comprehensively searching literature and conducting systematic reviews, two rounds of Delphi were conducted to reach consensus on the recommendations as follows: The histopathological specimens of PCNSL patients should be obtained as safely and comprehensively as possible by multimodal tomography-guided biopsy or minimally invasive surgery. Corticosteroids should be withdrawn from, or not be administered to, patients with suspected PCNSL before biopsy if the patient's status permits. MRI (enhanced and DWI) should be performed for diagnosing and evaluating PCNSL patients where whole-body PET-CT be used at necessary time points. Mini-mental status examination can be used to assess cognitive function in the clinical management. Newly diagnosed PCNSL patients should be treated with combined high-dose methotrexate-based regimen and can be treated with a rituximab-inclusive regimen at induction therapy. Autologous stem cell transplantation can be used as a consolidation therapy. Refractory or relapsed PCNSL patients can be treated with ibrutinib with or without high-dose chemotherapy as re-induction therapy. Stereotactic radiosurgery can be used for PCNSL patients with a limited recurrent lesion who were refractory to chemotherapy and have previously received whole-brain radiotherapy. Patients with suspected primary vitreoretinal lymphoma (PVRL) should be diagnosed by vitreous biopsy. PVRL or PCNSL patients with concurrent VRL can be treated with combined systemic and local therapy.

Keywords: China; Consensus; Management; Primary central nervous system lymphoma.

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Conflict of interest statement

All the authors and members declare that they have no competing interests.

Figures

**Fig. 1**
Flowchart of diagnosis and treatment. Common procedure of diagnosis, evaluation, and therapeutic regimens for newly diagnosed and relapsed/refractory PCNSL patients

**Fig. 2**
Link tables for the management of PCNSL. Additional information of preoperative steroids treatment, evaluation post-diagnosis, induction therapy, consolidation therapy, and treatment for relapsed/refractory patients

See this image and copyright information in PMC

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Evidence-based expert consensus on the management of primary central nervous system lymphoma in China

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Evidence-based expert consensus on the management of primary central nervous system lymphoma in China

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