Mycosis fongoïde avec transformation en un lymphome cutané pléiomorphe et localisation secondaire cérébrale

Guilloton, L.; Drouet, A.; Estival, J.-L.; Pierre, G. Saint; Dupin, M.; Ribot, C.

doi:10.1016/s0248-8663(01)00496-9

Cited by 7 publications

(1 citation statement)

References 18 publications

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“…CNS involvement is observed within an average of 3–5 years from the initial diagnosis, typically occurring in patients with advanced infiltration of other organs (Stein et al 2006), with autopsy demonstrating CNS involvement in 11–14% of cases (Zonenshayn et al; 1998). During life, CNS involvement is unusual, having been observed in 1.6% of 187 patients with cutaneous T-cell Lymphoma in one single series (Guilloton et al 2001). …”

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Central nervous system involvement in mycosis fungoides: relevance of tcr gene testing in cerebrospinal fluid

et al. 2014

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IntroductionMycosis Fungoides (MF) is a rare malignant T-cell lymphoma, involving mainly the skin. In 50%–75% of cases, it can involve organs other than skin, with a 11%–14% Central Nervous System involvement (CNS).Case reportA 82-year-old woman presented to our Department with a 15-years history of MF with skin lesions. Neurological examination showed dysarthria and a left facio-brachial-crural hemiparesis. A CT scan showed a right fronto-rolandic lesion. A MRI, including DWI, confirmed the presence of the “neoplastic” lesion with slight hemorrhagic component and leptomeningeal contrast enhancement. Molecular TCR rearrangement test by PCR analysis was performed on skin biopsy, showed the presence of a single peak which fits with a monoclonal TCRG gene rearrangement (size 67). Molecular TCR test was also performed on the cerebrospinal fluid (CSF), which confirmed the presence of lymphocyte clone T g/ more expressed with the same size of that observed in the skin biopsy A total body CT scan did not show any lymphnodal or extranodal disease. The patient died after ten days.ConclusionMF usually occurs in the context of advanced and often histologically transformed cutaneous disease. Isolated CNS involvement is remarkably rare. This case highlights the need for regular neurologic follow-up after the diagnosis of MF, in particular when features that suggest risk of disease progression are present. Furthermore, the analysis of the skin biopsy and above all of CSF by PCR technique, based on our experience, should always be executed in MF patients with signs or symptoms suggesting CNS involvement.

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Central nervous system involvement in mycosis fungoides: relevance of tcr gene testing in cerebrospinal fluid

et al. 2014

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Neurolymphomatosis Associated With Sézary Syndrome

Bézier

Réguiai²,

Delaby³

et al. 2009

Arch Dermatol

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Background: Mycosis fungoides and Sézary syndrome are cutaneous T-cell lymphomas characterized by the epidermotropism of tumor cells. Neuropathic disease is rare during mycosis fungoides and Sézary syndrome and usually results from a central nervous system involvement in late stages. Neurolymphomatosis is defined as the infiltration of the peripheral nerves by tumor lymphocytes. It has been described in patients with aggressive systemic lymphomas but, to our knowledge, not in patients with mycosis fungoides or Sézary syndrome. We report the first case of neurolymphomatosis in a patient with Sézary syndrome and the partial efficacy of highdose methotrexate sodium in treating this usually refractory complication.

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