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Primary central nervous system lymphoma

What's new

Added 2024 EHA/ESMO, 2023 EANO, and 2019 BSH guidelines for the diagnosis and management of 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

Surgical resection: as per EHA/ESMO 2024 guidelines, do not perform tumor resection except in carefully selected patients with rapidly increasing ICP likely to benefit from surgical debulking at the time of tumor biopsy.
D

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