【JHO】Chinese expert consensus on clinical practice of primary CNS lymphoma based on evidence

Primary central nervous system lymphoma (PCNSL) is a relatively rare subtype of lymphoma, the incidence of which accounts for about 1% of non-Hodgkin lymphoma and 3~4% of central nervous system tumors, of which 95% are diffuse large B-cell lymphomas
.
Because tumors are located in the central nervous system, conventional drugs are difficult to penetrate the blood-brain barrier, and tumor molecular classification is highly malignant, so its prognosis is far behind that of systemic lymphoma
other than CNS.
In addition, because the disease is relatively rare, medical workers in different regions have different understandings of the disease, and many patients are difficult to obtain standardized diagnosis and treatment
.
At present, there is relatively little expert consensus on the clinical practice of PCNSL and less specifically for this special disease, and there are no clinical practice guidelines or expert consensus
specifically for PCNSL in China.

Based on this, the Neurosurgery Branch of the Chinese Medical Association and the Hematology and Oncology Professional Committee of the Chinese Anti-Cancer Association jointly initiated, led by Huashan Hospital affiliated to Fudan University, and dozens of experts from different specialties (neurosurgery, hematology, neurology, ophthalmology, oncology, etc.
) from many hospitals in the United Nations formulated the "Chinese Expert Consensus on Clinical Practice of Primary Central Nervous System Lymphoma Based on Evidence", which was recently published in the Journal of Hematology & Oncology.
Professor Mao Ying, Department of Neurosurgery, Professor Chen Tong, Department of Hematology, Huashan Hospital, and Professor Chen Yaolong of Lanzhou University are co-corresponding authors, and Professor Chen Tong and first author
.
The main contents of this consensus are summarized as follows, and teachers are requested to criticize and correct
.

A summary of expert advice

Biopsy and diagnosis: it is recommended that histopathological specimens from patients with PCNSL should be obtained as safely and comprehensively as possible by multimodal tomography-guided biopsy or minimally invasive surgery (2C); Patients with suspected PCNSL should be discontinued or given glucocorticoids (1C) before biopsy if patient status allows; Patients with suspected PVRL should be discontinued from glucocorticoids (2D)
at least 2 weeks prior to biopsy if patient status allows.

Staging and follow-up evaluation: MRI (enhanced and DWI) is recommended for the diagnosis and evaluation of patients with PCNSL (1B); Systemic PET-CT is recommended to evaluate patients with PCNSL at specific time points, such as at initial diagnosis or relapse (2B); MMSE is recommended for assessing cognitive function in the management of patients with PCNSL (2B).

Induction therapy: if the patient is candidates for chemotherapy, patients with newly diagnosed PCNSL are advised to receive HD-MTX-containing combination therapy (1B), which can be rituximab (2C), cytarabine (2B), temozolomide (2C), or other drugs that can cross the blood-brain barrier (2C).

Patients with newly diagnosed PCNS-DLBCL are advised to receive a rituximab-containing regimen (2C)
at the time of induction therapy.

Consolidation therapy: ASCT is recommended as consolidation therapy (2C)
in patients with PCNSL who are candidates for preconditioning chemotherapy compared with non-tapering WBRT.

Refractory/relapse: patients with refractory/relapsed PCNSL are advised to receive ibrutinib with or without high-dose chemotherapy as reinduction therapy (2C); Stereotactic radiosurgery is recommended for patients with PCNSL (2D)
with localized relapsed lesions refractory to chemotherapy and who have previously received WBRT.

Intraocular involvement: it is recommended that patients with suspected PVRL should be diagnosed by vitreous biopsy (2D); Patients with PVRL or PCNSL with concurrent VRL are advised to receive a combination of systemic and local therapy (2C).

At baseline and follow-up, evaluation of neurocognitive function in patients with PCNSL is recommended

Suggested flow chart of diagnosis and treatment

References

Tong Chen, Yuanbo Liu, Yang Wang,et al.
Evidence-based expert consensus on the management of primary central nervous system lymphoma in China.
J Hematol Oncol .
2022 Sep 29; 15(1):136.
doi: 10.
1186/s13045-022-01356-7.

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