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Primary central nervous system lymphoma
Guidelines
Key sources
The following summarized guidelines for the evaluation and management of primary central nervous system lymphoma are prepared by our editorial team based on guidelines from the European Hematology Association (EHA/ESMO 2024), the European Association of Neuro-Oncology (EANO 2023), the British Society for Haematology (BSH 2019), the European Society of Medical Oncology (ESMO 2016), and the British HIV Association (BHIVA 2014).
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Diagnostic investigations
Diagnostic imaging
As per EHA/ESMO 2024 guidelines:
Obtain contrast-enhanced cranial MRI in patients with PCNSL.
B
Use the International PCNSL Collaborative Group protocol based on 3T or 1.5T MRI. B
Obtain spinal cord imaging in symptomatic patients or in case of CSF positivity.
B
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Imaging for staging
Ophthalmological assessment
Cognitive assessment
Diagnostic procedures
Biopsy and histopathology: as per EHA/ESMO 2024 guidelines, perform tumor biopsy and histopathological examination to confirm the diagnosis of PCNSL.
B
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Lumbar puncture
Vitreous sampling
Medical management
General principles: as per EHA/ESMO 2024 guidelines, offer enrollment in suitable prospective clinical trials to all patients with PCNSL.
A
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Induction chemotherapy (systemic)
Induction chemotherapy (intrathecal)
Consolidation therapy (whole-brain radiotherapy)
Induction chemotherapy (intravitreal)
Consolidation therapy (chemotherapy-ASCT)
Surgical interventions
Specific circumstances
Patients with primary vitreoretinal lymphoma: as per EHA/ESMO 2024 guidelines, offer high-dose methotrexate-based induction chemotherapy in fit patients with primary vitreoretinal lymphoma, using the same combinations proposed for other patients with PCNSL. Consider adding intravitreal methotrexate injections if a rapid regression of intraocular disease is needed.
B
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Patients with HIV-related PCNSL
Patients with post-transplant lymphoproliferative disorder
Follow-up and surveillance
Assessment of treatment response: as per EHA/ESMO 2024 guidelines, assess for treatment response according to modalities and timing proposed by the International PCNSL Collaborative Group criteria.
B
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Management of refractory/relapsed disease (evaluation)
Management of refractory/relapsed disease (management)
Follow-up