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03 Central Nervous System (CNS) Lymphoma

Revised 23 Oct.2002

1. Primary Parenchymal CNS Lymphoma
The diagnosis of primary CNS lymphoma should be based on biopsy of the lesion if possible. If the situation precludes biopsy and the diagnosis appears probable, evaluation and management should be discussed and planned with a medical oncology member of the Lymphoma Tumour Group at a BCCA Center. All patients with primary lymphoma of the CNS should have HIV antibody testing and a detailed ophthalmologic evaluation. Bone marrow biopsy and abdomino-pelvic CT scanning are not necessary in these patients. Because of poor long term disease control and significant neurotoxicity, the Lymphoma Tumour Group no longer recommends irradiation as the treatment of primary CNS lymphoma.
Treatment of patients with primary CNS lymphoma is based on neurologic and performance status after initial treatment with pharmacologic doses of corticosteroids (dexamethasone 8 to 16 mg orally each day). Patients who remain very frail, moribund or severely demented despite corticosteroids should not have their lymphoma further actively treated and care should concentrate on symptomatic palliation. All others should be offered treatment with high dose methotrexate (LY HD MTX) with leucovorin rescue. If renal dysfunction precludes high dose methotrexate whole brain plus posterior globe irradiation should be considered.
2. Leptomeningeal CNS Lymphoma
Lymphomatous involvement of the leptomeninges can occur due to spread from systemic or primary parenchymal CNS lymphoma. Diagnosis is based on CSF cytology and typical cranial and peripheral nerve abnormalities. Leptomeningeal lymphoma is very difficult to eradicate and reliable treatment has not been defined. High dose systemic methotrexate has been shown to provide superior palliation compared to intrathecal or intraventricular chemotherapy (Glantz, J Clin Oncol, 1998; 16: 1561-7). Patients with leptomeningeal lymphoma and adequate renal function (creatinine clearance >60ml/min) should be given high dose methotrexate with leucovorin rescue (LY HD MTX). Otherwise systemic high dose corticosteroids offer the best palliation.
3. CNS Prophylaxis

Patients with initial paranasal sinus involvement with aggressive lymphoma should receive methotrexate 12 mg alternating with cytarabine 50 mg intrathecally twice weekly x 6 doses (3 doses of each over 3 weeks) starting after completion of all other planned treatment and documentation of satisfactory response.


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Unofficial document if printed. Please refer to the following web address for up-to-date information: http://www.bccancer.bc.ca/HPI/CancerManagementGuidelines/Lymphoma/HD/03CentralNervousSystem%28CNS%29.htm