Temozolomide for Central Nervous System Involvement in Mycosis Fungoides

Background The central nervous system (CNS) is one of the most commonly involved sites in the systemic progression of primary cutaneous T cell lymphomas (CTCLs) such as mycosis fungoides (MF). There is no consensus on the treatment of CNS progression in CTCL, but survival of 3-6 months is suggested when methotrexate-based chemotherapy and/or CNS irradiation is used. Temozolomide is active in earlier stages of MF and readily crosses the blood-brain barrier. There are no published data on its use in MF patients with CNS involvement.Methods Four MF patients were treated with oral temozolomide (200 mg/m(2) per day for 5 d on a 28-day cycle) for CNS progression. Two patients received temozolomide with low-dose CNS irradiation as initial treatment, and two received temozolomide following disease progression after methotrexate-based chemotherapy and CNS irradiation. All patients received dexamethasone.Results Temozolomide was well tolerated; there were no treatment withdrawals or dose reductions caused by toxicity. Patient 1 had an excellent partial response in pre-irradiated disease. Patient 2 showed disease stabilization following irradiation. Patient 3 showed a complete response after a partial response to irradiation. Patient 4 demonstrated continued stabilization after a partial response to irradiation. Overall survival ranged from 10 to 33 months. Patient 3 remains alive and symptom-free at 23 months following treatment.Conclusions Temozolomide following low-dose CNS irradiation appears to be well tolerated and effective in MF patients with CNS progression. It may represent a less toxic alternative to chemotherapy containing methotrexate or an option for second-line therapy.