“…Involvement of the central nervous system is a rare complication but has been reported in 11%–14% of cases 10. Examples of such involvement have been seen in association with the tumor‐stage of mycosis fungoides with involvement of the leptomeninges, medulla, spinal cord, and cranial nerves 5, 7, 19–21, 35, 40. Peripheral nerve involvement, which is rare, is caused by direct infiltration,9 a “remote” noninfiltrative mechanism,6 or transformation of mycosis fungoides into large T‐cell lymphoma with subsequent infiltration of nerves 28.…”
Occasional cases of peripheral neuropathy have been reported in classic mycosis fungoides. A rare variant of mycosis fungoides is the granulomatous form. We describe the occurrence of myopathy and peripheral neuropathy in a young woman who had skin lesions since the age of 12 years. At the age of 20 years they were diagnosed as granulomatous mycosis fungoides. The skin lesions resolved with interferon therapy and radiation. She then presented with cardiac and pulmonary symptoms and signs that were initially thought to be due to sarcoidosis or systemic vasculitis. A nerve and muscle biopsy showed granulomatous mycosis fungoides. To our knowledge, involvement of muscle and nerve by granulomatous mycosis fungoides has not been reported previously. Early reports suggested a favorable prognosis for the granulomatous subtype of mycosis fungoides. Based on a literature review and the course in our case, however, granulomatous mycosis fungoides seems to be an indicator of aggressive disease and ultimately a poor prognosis.
“…Involvement of the central nervous system is a rare complication but has been reported in 11%–14% of cases 10. Examples of such involvement have been seen in association with the tumor‐stage of mycosis fungoides with involvement of the leptomeninges, medulla, spinal cord, and cranial nerves 5, 7, 19–21, 35, 40. Peripheral nerve involvement, which is rare, is caused by direct infiltration,9 a “remote” noninfiltrative mechanism,6 or transformation of mycosis fungoides into large T‐cell lymphoma with subsequent infiltration of nerves 28.…”
Occasional cases of peripheral neuropathy have been reported in classic mycosis fungoides. A rare variant of mycosis fungoides is the granulomatous form. We describe the occurrence of myopathy and peripheral neuropathy in a young woman who had skin lesions since the age of 12 years. At the age of 20 years they were diagnosed as granulomatous mycosis fungoides. The skin lesions resolved with interferon therapy and radiation. She then presented with cardiac and pulmonary symptoms and signs that were initially thought to be due to sarcoidosis or systemic vasculitis. A nerve and muscle biopsy showed granulomatous mycosis fungoides. To our knowledge, involvement of muscle and nerve by granulomatous mycosis fungoides has not been reported previously. Early reports suggested a favorable prognosis for the granulomatous subtype of mycosis fungoides. Based on a literature review and the course in our case, however, granulomatous mycosis fungoides seems to be an indicator of aggressive disease and ultimately a poor prognosis.
“…Aside from case reports, there is no published evidence on the management of CNS disease in MF. Approximately 60 cases have been published in the literature, but many of these patients did not receive any active treatment with chemotherapy or radiation. A case series by Stein et al .…”
Section: Discussionmentioning
confidence: 99%
“…1,6 Central nervous system involvement is a devastating complication of MF. Approximately 60 cases have been reported in the literature, 5,[7][8][9][10][11] with survival generally being in the region of 3-6 months. There are no published studies investigating the treatment of CNS involvement in MF.…”
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.
To determine some of the key clinical features that help prompt clinicians to pursue additional work-up for evaluation of CNS involvement of MF, we conducted a systematic review to better define characteristics, treatments, outcomes, and mortality in these patients. Our analyses indicated that neurologic surveillance after the diagnosis of MF is crucial. Review of systems should include change in mentation, vestibular, and ocular symptoms. Progression to CNS involvement does not always occur in tandem with cutaneous disease burden. Single-agent therapies can delay disease progression and improve prognosis. Multi-agent treatment does not improve survival.
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