Diagnosis and management of primary central nervous system lymphoma

Abstract

Primary central nervous system lymphoma (PCNSL) is a rare and aggressive extranodal non-Hodgkin lymphoma (NHL) that is confined to the brain, eyes, spinal cord, or leptomeninges without systemic involvement. The overall prognosis, diagnosis, and management of PCNSL differ from those for other types of NHL. Prompt diagnosis and initiation of treatment are vital for improving clinical outcomes. PCNSL is responsive to radiation therapy; however, whole-brain radiotherapy (WBRT) inadequately controls the disease when it is used alone, and its delayed neurotoxicity causes neurocognitive impairment, especially in elderly patients. High-dose methotrexate (HD-MTX)-based induction chemotherapy with or without autologous stem cell transplantation (ASCT) or reduced-dose WBRT leads to durable disease control and less neurotoxicity. The optimal treatment has yet to be defined; however, HD-MTX-based induction chemotherapy is considered standard for newly diagnosed PCNSL. Ongoing randomized trials are addressing the roles of rituximab and consolidative treatment with ASCT or reduced-dose WBRT. Despite high tumor response rates with the initial treatment, many patients relapse with a very poor prognosis. The optimal treatment for refractory or relapsed PCNSL is poorly defined. The choice of salvage treatment depends on a patient's age, previous treatment and response, performance status, and comorbidities at the time of relapse. This review provides an overview of the clinical features, diagnosis, pathology, and management of PCNSL in immunocompetent patients, and it focuses on recent advances in treatment. Cancer 2017;123:4314-24. © 2017 American Cancer Society.

5-ALA–induced fluorescence as a marker for diagnostic tissue in stereotactic biopsies of intracranial lymphomas: experience in 41 patients

“…1,22 Generally, the treatment of intracranial lymphomas consists of high-dose methotrexate chemotherapy after histopathological confirmation by neurosurgical biopsy. 11 Nowadays, navigation-guided frameless stereotactic needle biopsies are frequently performed due to the minimal invasiveness of this procedure. 3,7,11 Whenever clinically possible, patients with suspected intracranial lymphoma should not receive preoperative corticosteroid treatment to prevent an inaccurate histopathological diagnosis.…”

“…11 Nowadays, navigation-guided frameless stereotactic needle biopsies are frequently performed due to the minimal invasiveness of this procedure. 3,7,11 Whenever clinically possible, patients with suspected intracranial lymphoma should not receive preoperative corticosteroid treatment to prevent an inaccurate histopathological diagnosis. 11,22 Although stereotactic lymphoma biopsy constitutes a widely performed standard neurosurgical procedure, it is associated with specific limitations.…”

“…3,7,11 Whenever clinically possible, patients with suspected intracranial lymphoma should not receive preoperative corticosteroid treatment to prevent an inaccurate histopathological diagnosis. 11,22 Although stereotactic lymphoma biopsy constitutes a widely performed standard neurosurgical procedure, it is associated with specific limitations. In this sense, the most important limitation constitutes the acquisition of nondiagnostic biopsy samples, which is reported in up to 29% of cases.…”

Intraocular chemotherapy for vitreoretinal lymphoma: A review

“…In targeting B lymphocytes, rituximab effects depletion of the B lymphocytes by multiple mechanisms that include antibody‐dependent cellular cytotoxicity, complement‐dependent cytotoxicity and lymphocyte apoptosis. The drug is used systemically in the treatment of a range of B lymphocyte‐mediated diseases: autoimmune diseases, including some forms of uveitis; and B‐cell lymphomas, including PCNSL . The first reported uses of intraocular rituximab therapy for VRL were in 2007, by Kitzmann et al from Mayo Clinic (Rochester, MN) and Ohgura et al from Osaka University Medical School (Osaka, Japan): a total of eight eyes of five patients were treated with up to four intravitreal injections of rituximab.…”

“…The drug is used systemically in the treatment of a range of B lymphocyte-mediated diseases: autoimmune diseases, including some forms of uveitis; 53 and B-cell lymphomas, including PCNSL. 54 The first reported uses of intraocular rituximab therapy for VRL were in 2007, by Kitzmann et al from Mayo Clinic (Rochester, MN) 18 and Ohgura et al from Osaka University Medical School (Osaka, Japan): 55 a total of eight eyes of five patients were treated with up to four intravitreal injections of rituximab. The follow-up intervals were short, but all injected eyes showed partial or complete remission of VRL following the treatment, which also was well tolerated.…”