Primary CNS lymphoma

Tracy Batchelor1, Jay S Loeffler

  • 1Stephen E. and Catherine Pappas Center for Neuro-Oncology, Massachusetts General Hospital, Boston, MA 02114, USA. tbatchelor@partners.org

Insights

Primary CNS lymphoma (PCNSL) management requires neurosurgical consultation and biopsy. Chemotherapy with or without radiation improves outcomes, but chemotherapy alone may reduce neurotoxicity risks.

Area of Science:

  • Neuro-oncology
  • Hematology
  • Oncology

Background:

  • Primary CNS lymphoma (PCNSL) is a rare non-Hodgkin's lymphoma (NHL) affecting the brain, spinal cord, or eyes.
  • PCNSL incidence has risen, impacting both immunocompromised and immunocompetent individuals.
  • Management differs from other extranodal NHLs, presenting unique diagnostic and therapeutic challenges.

Purpose of the Study:

  • To outline diagnostic and therapeutic strategies for primary CNS lymphoma (PCNSL) in immunocompetent patients.
  • To review the role of neuroimaging, biopsy, and systemic staging in PCNSL diagnosis.
  • To evaluate treatment modalities including surgery, radiation, and chemotherapy for PCNSL.

Main Methods:

  • Characteristic imaging features suggestive of PCNSL.
  • Neurosurgical consultation for stereotactic biopsy.
  • Assessment of brain, cerebrospinal fluid (CSF), and eyes for disease involvement.
  • Screening for occult systemic disease.
  • Evaluation of treatment outcomes for surgery, whole-brain radiation therapy (WBRT), and chemotherapy.

Main Results:

  • Surgical resection offers no therapeutic benefit, reserved only for herniation.
  • WBRT alone is insufficient for durable control and carries high neurotoxicity risk in older adults.
  • Combined chemotherapy and WBRT improve response rates and survival compared to WBRT alone.
  • Methotrexate-based chemotherapy without WBRT shows comparable efficacy and potentially lower neurotoxicity.

Conclusions:

  • Early diagnosis and neurosurgical consultation are crucial for PCNSL management.
  • Chemotherapy, particularly methotrexate-based regimens, is a cornerstone of treatment, with or without WBRT.
  • Balancing efficacy and neurotoxicity is key, favoring chemotherapy-alone approaches in select cases to preserve quality of life.

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