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

Introduction Mycosis 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 report A 82-year-old woman presented to our Department with a 15-years history...
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Gespeichert in:

Autor*in:	Giorli, Elisa [verfasserIn] Traverso, Elisabetta [verfasserIn] Benedetti, Luana [verfasserIn] Zupo, Simona [verfasserIn] Del Sette, Bruno [verfasserIn] Cerruti, Giannamaria [verfasserIn] Godani, Massimiliano [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2014

Schlagwörter:	Mycosis fungoides CNS involvement PCR Cerebrospinal fluid CSF PCR analisys

Übergeordnetes Werk:	Enthalten in: SpringerPlus - London : Biomed Central, 2012, 3(2014), 1 vom: 17. Jan.
Übergeordnetes Werk:	volume:3 ; year:2014 ; number:1 ; day:17 ; month:01

Links:	Volltext

DOI / URN:	10.1186/2193-1801-3-29

Katalog-ID:	SPR032761082

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520			\|a Introduction Mycosis 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 report A 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. Conclusion MF 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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allfields	10.1186/2193-1801-3-29 doi (DE-627)SPR032761082 (SPR)2193-1801-3-29-e DE-627 ger DE-627 rakwb eng 600 ASE Giorli, Elisa verfasserin aut Central nervous system involvement in mycosis fungoides: relevance of tcr gene testing in cerebrospinal fluid 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Introduction Mycosis 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 report A 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. Conclusion MF 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. Mycosis fungoides (dpeaa)DE-He213 CNS involvement (dpeaa)DE-He213 PCR (dpeaa)DE-He213 Cerebrospinal fluid (dpeaa)DE-He213 CSF (dpeaa)DE-He213 PCR analisys (dpeaa)DE-He213 Traverso, Elisabetta verfasserin aut Benedetti, Luana verfasserin aut Zupo, Simona verfasserin aut Del Sette, Bruno verfasserin aut Cerruti, Giannamaria verfasserin aut Godani, Massimiliano verfasserin aut Enthalten in SpringerPlus London : Biomed Central, 2012 3(2014), 1 vom: 17. Jan. (DE-627)718615298 (DE-600)2661116-8 2193-1801 nnns volume:3 year:2014 number:1 day:17 month:01 https://dx.doi.org/10.1186/2193-1801-3-29 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4335 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 3 2014 1 17 01
spelling	10.1186/2193-1801-3-29 doi (DE-627)SPR032761082 (SPR)2193-1801-3-29-e DE-627 ger DE-627 rakwb eng 600 ASE Giorli, Elisa verfasserin aut Central nervous system involvement in mycosis fungoides: relevance of tcr gene testing in cerebrospinal fluid 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Introduction Mycosis 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 report A 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. Conclusion MF 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. Mycosis fungoides (dpeaa)DE-He213 CNS involvement (dpeaa)DE-He213 PCR (dpeaa)DE-He213 Cerebrospinal fluid (dpeaa)DE-He213 CSF (dpeaa)DE-He213 PCR analisys (dpeaa)DE-He213 Traverso, Elisabetta verfasserin aut Benedetti, Luana verfasserin aut Zupo, Simona verfasserin aut Del Sette, Bruno verfasserin aut Cerruti, Giannamaria verfasserin aut Godani, Massimiliano verfasserin aut Enthalten in SpringerPlus London : Biomed Central, 2012 3(2014), 1 vom: 17. Jan. (DE-627)718615298 (DE-600)2661116-8 2193-1801 nnns volume:3 year:2014 number:1 day:17 month:01 https://dx.doi.org/10.1186/2193-1801-3-29 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4335 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 3 2014 1 17 01
allfields_unstemmed	10.1186/2193-1801-3-29 doi (DE-627)SPR032761082 (SPR)2193-1801-3-29-e DE-627 ger DE-627 rakwb eng 600 ASE Giorli, Elisa verfasserin aut Central nervous system involvement in mycosis fungoides: relevance of tcr gene testing in cerebrospinal fluid 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Introduction Mycosis 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 report A 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. Conclusion MF 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. Mycosis fungoides (dpeaa)DE-He213 CNS involvement (dpeaa)DE-He213 PCR (dpeaa)DE-He213 Cerebrospinal fluid (dpeaa)DE-He213 CSF (dpeaa)DE-He213 PCR analisys (dpeaa)DE-He213 Traverso, Elisabetta verfasserin aut Benedetti, Luana verfasserin aut Zupo, Simona verfasserin aut Del Sette, Bruno verfasserin aut Cerruti, Giannamaria verfasserin aut Godani, Massimiliano verfasserin aut Enthalten in SpringerPlus London : Biomed Central, 2012 3(2014), 1 vom: 17. Jan. (DE-627)718615298 (DE-600)2661116-8 2193-1801 nnns volume:3 year:2014 number:1 day:17 month:01 https://dx.doi.org/10.1186/2193-1801-3-29 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4335 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 3 2014 1 17 01
allfieldsGer	10.1186/2193-1801-3-29 doi (DE-627)SPR032761082 (SPR)2193-1801-3-29-e DE-627 ger DE-627 rakwb eng 600 ASE Giorli, Elisa verfasserin aut Central nervous system involvement in mycosis fungoides: relevance of tcr gene testing in cerebrospinal fluid 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Introduction Mycosis 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 report A 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. Conclusion MF 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. Mycosis fungoides (dpeaa)DE-He213 CNS involvement (dpeaa)DE-He213 PCR (dpeaa)DE-He213 Cerebrospinal fluid (dpeaa)DE-He213 CSF (dpeaa)DE-He213 PCR analisys (dpeaa)DE-He213 Traverso, Elisabetta verfasserin aut Benedetti, Luana verfasserin aut Zupo, Simona verfasserin aut Del Sette, Bruno verfasserin aut Cerruti, Giannamaria verfasserin aut Godani, Massimiliano verfasserin aut Enthalten in SpringerPlus London : Biomed Central, 2012 3(2014), 1 vom: 17. Jan. (DE-627)718615298 (DE-600)2661116-8 2193-1801 nnns volume:3 year:2014 number:1 day:17 month:01 https://dx.doi.org/10.1186/2193-1801-3-29 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4335 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 3 2014 1 17 01
allfieldsSound	10.1186/2193-1801-3-29 doi (DE-627)SPR032761082 (SPR)2193-1801-3-29-e DE-627 ger DE-627 rakwb eng 600 ASE Giorli, Elisa verfasserin aut Central nervous system involvement in mycosis fungoides: relevance of tcr gene testing in cerebrospinal fluid 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Introduction Mycosis 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 report A 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. Conclusion MF 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. Mycosis fungoides (dpeaa)DE-He213 CNS involvement (dpeaa)DE-He213 PCR (dpeaa)DE-He213 Cerebrospinal fluid (dpeaa)DE-He213 CSF (dpeaa)DE-He213 PCR analisys (dpeaa)DE-He213 Traverso, Elisabetta verfasserin aut Benedetti, Luana verfasserin aut Zupo, Simona verfasserin aut Del Sette, Bruno verfasserin aut Cerruti, Giannamaria verfasserin aut Godani, Massimiliano verfasserin aut Enthalten in SpringerPlus London : Biomed Central, 2012 3(2014), 1 vom: 17. Jan. (DE-627)718615298 (DE-600)2661116-8 2193-1801 nnns volume:3 year:2014 number:1 day:17 month:01 https://dx.doi.org/10.1186/2193-1801-3-29 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4335 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 3 2014 1 17 01
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In 50%–75% of cases, it can involve organs other than skin, with a 11%–14% Central Nervous System involvement (CNS). Case report A 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. Conclusion MF 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. 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author	Giorli, Elisa
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topic_title	600 ASE Central nervous system involvement in mycosis fungoides: relevance of tcr gene testing in cerebrospinal fluid Mycosis fungoides (dpeaa)DE-He213 CNS involvement (dpeaa)DE-He213 PCR (dpeaa)DE-He213 Cerebrospinal fluid (dpeaa)DE-He213 CSF (dpeaa)DE-He213 PCR analisys (dpeaa)DE-He213
topic	ddc 600 misc Mycosis fungoides misc CNS involvement misc PCR misc Cerebrospinal fluid misc CSF misc PCR analisys
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title	Central nervous system involvement in mycosis fungoides: relevance of tcr gene testing in cerebrospinal fluid
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title_full	Central nervous system involvement in mycosis fungoides: relevance of tcr gene testing in cerebrospinal fluid
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title_sort	central nervous system involvement in mycosis fungoides: relevance of tcr gene testing in cerebrospinal fluid
title_auth	Central nervous system involvement in mycosis fungoides: relevance of tcr gene testing in cerebrospinal fluid
abstract	Introduction Mycosis 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 report A 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. Conclusion MF 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.
abstractGer	Introduction Mycosis 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 report A 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. Conclusion MF 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.
abstract_unstemmed	Introduction Mycosis 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 report A 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. Conclusion MF 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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title_short	Central nervous system involvement in mycosis fungoides: relevance of tcr gene testing in cerebrospinal fluid
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author2	Traverso, Elisabetta Benedetti, Luana Zupo, Simona Del Sette, Bruno Cerruti, Giannamaria Godani, Massimiliano
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up_date	2024-07-03T14:38:31.176Z
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In 50%–75% of cases, it can involve organs other than skin, with a 11%–14% Central Nervous System involvement (CNS). Case report A 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. Conclusion MF 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. 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Central nervous system involvement in mycosis fungoides: relevance of tcr gene testing in cerebrospinal fluid

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