Symptoms associated with malignancy of peripheral nerve sheath tumours: a retrospective study of 69 patients with neurofibromatosis 1
Summary Background Neurofibromatosis 1 (NF1) is a common genetic disorder with variable clinical manifestations and an unpredictable course. Plexiform neurofibromas are common complications of NF1. Their malignant transformation is the main cause of mortality in adult patients with NF1. Objectives ...
Gespeichert in:
| Veröffentlicht in: | British journal of dermatology (1951) 2005-07, Vol.153 (1), p.79-82 |
|---|---|
| Hauptverfasser: | , , , , , , |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | 82 |
|---|---|
| container_issue | 1 |
| container_start_page | 79 |
| container_title | British journal of dermatology (1951) |
| container_volume | 153 |
| creator | Valeyrie-Allanore, L. Ismaïli, N. Bastuji-Garin, S. Zeller, J. Wechsler, J. Revuz, J. Wolkenstein, P. |
| description | Summary
Background Neurofibromatosis 1 (NF1) is a common genetic disorder with variable clinical manifestations and an unpredictable course. Plexiform neurofibromas are common complications of NF1. Their malignant transformation is the main cause of mortality in adult patients with NF1.
Objectives To identify clinical factors associated with malignant transformation of plexiform neurofibromas.
Methods Using the database of our neurofibromatosis clinic we included in a retrospective study all patients with NF1 having at least one peripheral nerve sheath tumour for which they underwent surgery or surgical biopsy. Predictive values for malignant transformation of three clinical symptoms, i.e. pain, enlargement of mass and neurological symptoms, were evaluated in association with histological parameters.
Results Of 69 patients studied, 48 had at least one plexiform neurofibroma and 21 had a malignant peripheral nerve sheath tumour. Only enlargement of the tumour had high negative and positive predictive values for malignant transformation: 0·92 and 0·95, respectively. In multivariate analysis, tumour enlargement was independently associated with malignant transformation (odds ratio 167·8, 95% confidence interval 14·0–2012·1).
Conclusions From a practical point of view, pain, neurological deficit and enlargement of a pre‐existing peripheral nerve sheath tumour in NF1 must lead to deep surgical biopsy to rule out malignant transformation. |
| doi_str_mv | 10.1111/j.1365-2133.2005.06558.x |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_68055810</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>68055810</sourcerecordid><originalsourceid>FETCH-LOGICAL-c4628-c5355bd9cffe92e71134435b48a618ddccf27d454ee959009dbd75f79bafaf413</originalsourceid><addsrcrecordid>eNqNkd1u1DAQhS0EokvhFZCFBHcJdhw7CRIXZYEtqAJV_EncWI4zZr3kD9uhuw_Ae-M0q4K4wjdjab5zNDMHIUxJSuN7ukspEzzJKGNpRghPieC8TPe30OqmcRutCCFFQirBTtA973eEUEY4uYtOqCBZxRhZoV8fDt0Yhs5j5f2grQrQ4CsbtrhTrf3Wq14f8GDwCM6OW3CqxT24n4D9FlSkwtQNk_PPsMIOghv8CDrYuR-m5lopKjyqYKEPfjHuYXKDsbUbOhUGbz2m99Edo1oPD471FH16_erj-jy5eL95sz67SHQusjLRnHFeN5U2BqoMCkpZnjNe56UStGwarU1WNDnPASpeEVI1dVNwU1S1MsrklJ2iJ4vv6IYfE_ggO-s1tK3qYZi8FCWJd6Qkgo_-AXdxzT7OJuO9qWC8mN3KBdJxb-_AyNHZTrmDpETOOcmdnOOQcxyzjsvrnOQ-Sh8e_ae6g-aP8BhMBB4fAeW1ao2LQVj_F1dlpSA8cs8X7sq2cPjvAeSLty_nX9Qni976APsbvXLfpShYweWXdxt5vt58vbzMPkvGfgPNKb-3</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>200163571</pqid></control><display><type>article</type><title>Symptoms associated with malignancy of peripheral nerve sheath tumours: a retrospective study of 69 patients with neurofibromatosis 1</title><source>MEDLINE</source><source>Oxford University Press Journals All Titles (1996-Current)</source><source>Wiley Online Library All Journals</source><creator>Valeyrie-Allanore, L. ; Ismaïli, N. ; Bastuji-Garin, S. ; Zeller, J. ; Wechsler, J. ; Revuz, J. ; Wolkenstein, P.</creator><creatorcontrib>Valeyrie-Allanore, L. ; Ismaïli, N. ; Bastuji-Garin, S. ; Zeller, J. ; Wechsler, J. ; Revuz, J. ; Wolkenstein, P.</creatorcontrib><description>Summary
Background Neurofibromatosis 1 (NF1) is a common genetic disorder with variable clinical manifestations and an unpredictable course. Plexiform neurofibromas are common complications of NF1. Their malignant transformation is the main cause of mortality in adult patients with NF1.
Objectives To identify clinical factors associated with malignant transformation of plexiform neurofibromas.
Methods Using the database of our neurofibromatosis clinic we included in a retrospective study all patients with NF1 having at least one peripheral nerve sheath tumour for which they underwent surgery or surgical biopsy. Predictive values for malignant transformation of three clinical symptoms, i.e. pain, enlargement of mass and neurological symptoms, were evaluated in association with histological parameters.
Results Of 69 patients studied, 48 had at least one plexiform neurofibroma and 21 had a malignant peripheral nerve sheath tumour. Only enlargement of the tumour had high negative and positive predictive values for malignant transformation: 0·92 and 0·95, respectively. In multivariate analysis, tumour enlargement was independently associated with malignant transformation (odds ratio 167·8, 95% confidence interval 14·0–2012·1).
Conclusions From a practical point of view, pain, neurological deficit and enlargement of a pre‐existing peripheral nerve sheath tumour in NF1 must lead to deep surgical biopsy to rule out malignant transformation.</description><identifier>ISSN: 0007-0963</identifier><identifier>EISSN: 1365-2133</identifier><identifier>DOI: 10.1111/j.1365-2133.2005.06558.x</identifier><identifier>PMID: 16029330</identifier><identifier>CODEN: BJDEAZ</identifier><language>eng</language><publisher>Oxford, UK: Blackwell Science Ltd</publisher><subject>Adolescent ; Adult ; Aged ; Biological and medical sciences ; Disease Progression ; enlargement ; Facial Neoplasms - pathology ; Female ; Humans ; Male ; malignant transformation ; Medical sciences ; Middle Aged ; Nerve Sheath Neoplasms - complications ; Nerve Sheath Neoplasms - pathology ; Nervous System Diseases - etiology ; neurofibromatosis 1 ; Neurofibromatosis 1 - complications ; Neurofibromatosis 1 - pathology ; Neurology ; Pain - etiology ; Retrospective Studies ; Risk Factors ; Tumors of the nervous system. Phacomatoses</subject><ispartof>British journal of dermatology (1951), 2005-07, Vol.153 (1), p.79-82</ispartof><rights>2005 INIST-CNRS</rights><rights>Copyright Blackwell Publishing Jul 2005</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4628-c5355bd9cffe92e71134435b48a618ddccf27d454ee959009dbd75f79bafaf413</citedby><cites>FETCH-LOGICAL-c4628-c5355bd9cffe92e71134435b48a618ddccf27d454ee959009dbd75f79bafaf413</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fj.1365-2133.2005.06558.x$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fj.1365-2133.2005.06558.x$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,780,784,1416,27922,27923,45572,45573</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=16928605$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/16029330$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Valeyrie-Allanore, L.</creatorcontrib><creatorcontrib>Ismaïli, N.</creatorcontrib><creatorcontrib>Bastuji-Garin, S.</creatorcontrib><creatorcontrib>Zeller, J.</creatorcontrib><creatorcontrib>Wechsler, J.</creatorcontrib><creatorcontrib>Revuz, J.</creatorcontrib><creatorcontrib>Wolkenstein, P.</creatorcontrib><title>Symptoms associated with malignancy of peripheral nerve sheath tumours: a retrospective study of 69 patients with neurofibromatosis 1</title><title>British journal of dermatology (1951)</title><addtitle>Br J Dermatol</addtitle><description>Summary
Background Neurofibromatosis 1 (NF1) is a common genetic disorder with variable clinical manifestations and an unpredictable course. Plexiform neurofibromas are common complications of NF1. Their malignant transformation is the main cause of mortality in adult patients with NF1.
Objectives To identify clinical factors associated with malignant transformation of plexiform neurofibromas.
Methods Using the database of our neurofibromatosis clinic we included in a retrospective study all patients with NF1 having at least one peripheral nerve sheath tumour for which they underwent surgery or surgical biopsy. Predictive values for malignant transformation of three clinical symptoms, i.e. pain, enlargement of mass and neurological symptoms, were evaluated in association with histological parameters.
Results Of 69 patients studied, 48 had at least one plexiform neurofibroma and 21 had a malignant peripheral nerve sheath tumour. Only enlargement of the tumour had high negative and positive predictive values for malignant transformation: 0·92 and 0·95, respectively. In multivariate analysis, tumour enlargement was independently associated with malignant transformation (odds ratio 167·8, 95% confidence interval 14·0–2012·1).
Conclusions From a practical point of view, pain, neurological deficit and enlargement of a pre‐existing peripheral nerve sheath tumour in NF1 must lead to deep surgical biopsy to rule out malignant transformation.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Aged</subject><subject>Biological and medical sciences</subject><subject>Disease Progression</subject><subject>enlargement</subject><subject>Facial Neoplasms - pathology</subject><subject>Female</subject><subject>Humans</subject><subject>Male</subject><subject>malignant transformation</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>Nerve Sheath Neoplasms - complications</subject><subject>Nerve Sheath Neoplasms - pathology</subject><subject>Nervous System Diseases - etiology</subject><subject>neurofibromatosis 1</subject><subject>Neurofibromatosis 1 - complications</subject><subject>Neurofibromatosis 1 - pathology</subject><subject>Neurology</subject><subject>Pain - etiology</subject><subject>Retrospective Studies</subject><subject>Risk Factors</subject><subject>Tumors of the nervous system. Phacomatoses</subject><issn>0007-0963</issn><issn>1365-2133</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2005</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkd1u1DAQhS0EokvhFZCFBHcJdhw7CRIXZYEtqAJV_EncWI4zZr3kD9uhuw_Ae-M0q4K4wjdjab5zNDMHIUxJSuN7ukspEzzJKGNpRghPieC8TPe30OqmcRutCCFFQirBTtA973eEUEY4uYtOqCBZxRhZoV8fDt0Yhs5j5f2grQrQ4CsbtrhTrf3Wq14f8GDwCM6OW3CqxT24n4D9FlSkwtQNk_PPsMIOghv8CDrYuR-m5lopKjyqYKEPfjHuYXKDsbUbOhUGbz2m99Edo1oPD471FH16_erj-jy5eL95sz67SHQusjLRnHFeN5U2BqoMCkpZnjNe56UStGwarU1WNDnPASpeEVI1dVNwU1S1MsrklJ2iJ4vv6IYfE_ggO-s1tK3qYZi8FCWJd6Qkgo_-AXdxzT7OJuO9qWC8mN3KBdJxb-_AyNHZTrmDpETOOcmdnOOQcxyzjsvrnOQ-Sh8e_ae6g-aP8BhMBB4fAeW1ao2LQVj_F1dlpSA8cs8X7sq2cPjvAeSLty_nX9Qni976APsbvXLfpShYweWXdxt5vt58vbzMPkvGfgPNKb-3</recordid><startdate>200507</startdate><enddate>200507</enddate><creator>Valeyrie-Allanore, L.</creator><creator>Ismaïli, N.</creator><creator>Bastuji-Garin, S.</creator><creator>Zeller, J.</creator><creator>Wechsler, J.</creator><creator>Revuz, J.</creator><creator>Wolkenstein, P.</creator><general>Blackwell Science Ltd</general><general>Blackwell</general><general>Oxford University Press</general><scope>BSCLL</scope><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>H94</scope><scope>K9.</scope><scope>NAPCQ</scope><scope>7X8</scope></search><sort><creationdate>200507</creationdate><title>Symptoms associated with malignancy of peripheral nerve sheath tumours: a retrospective study of 69 patients with neurofibromatosis 1</title><author>Valeyrie-Allanore, L. ; Ismaïli, N. ; Bastuji-Garin, S. ; Zeller, J. ; Wechsler, J. ; Revuz, J. ; Wolkenstein, P.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4628-c5355bd9cffe92e71134435b48a618ddccf27d454ee959009dbd75f79bafaf413</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2005</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Aged</topic><topic>Biological and medical sciences</topic><topic>Disease Progression</topic><topic>enlargement</topic><topic>Facial Neoplasms - pathology</topic><topic>Female</topic><topic>Humans</topic><topic>Male</topic><topic>malignant transformation</topic><topic>Medical sciences</topic><topic>Middle Aged</topic><topic>Nerve Sheath Neoplasms - complications</topic><topic>Nerve Sheath Neoplasms - pathology</topic><topic>Nervous System Diseases - etiology</topic><topic>neurofibromatosis 1</topic><topic>Neurofibromatosis 1 - complications</topic><topic>Neurofibromatosis 1 - pathology</topic><topic>Neurology</topic><topic>Pain - etiology</topic><topic>Retrospective Studies</topic><topic>Risk Factors</topic><topic>Tumors of the nervous system. Phacomatoses</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Valeyrie-Allanore, L.</creatorcontrib><creatorcontrib>Ismaïli, N.</creatorcontrib><creatorcontrib>Bastuji-Garin, S.</creatorcontrib><creatorcontrib>Zeller, J.</creatorcontrib><creatorcontrib>Wechsler, J.</creatorcontrib><creatorcontrib>Revuz, J.</creatorcontrib><creatorcontrib>Wolkenstein, P.</creatorcontrib><collection>Istex</collection><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Premium</collection><collection>MEDLINE - Academic</collection><jtitle>British journal of dermatology (1951)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Valeyrie-Allanore, L.</au><au>Ismaïli, N.</au><au>Bastuji-Garin, S.</au><au>Zeller, J.</au><au>Wechsler, J.</au><au>Revuz, J.</au><au>Wolkenstein, P.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Symptoms associated with malignancy of peripheral nerve sheath tumours: a retrospective study of 69 patients with neurofibromatosis 1</atitle><jtitle>British journal of dermatology (1951)</jtitle><addtitle>Br J Dermatol</addtitle><date>2005-07</date><risdate>2005</risdate><volume>153</volume><issue>1</issue><spage>79</spage><epage>82</epage><pages>79-82</pages><issn>0007-0963</issn><eissn>1365-2133</eissn><coden>BJDEAZ</coden><abstract>Summary
Background Neurofibromatosis 1 (NF1) is a common genetic disorder with variable clinical manifestations and an unpredictable course. Plexiform neurofibromas are common complications of NF1. Their malignant transformation is the main cause of mortality in adult patients with NF1.
Objectives To identify clinical factors associated with malignant transformation of plexiform neurofibromas.
Methods Using the database of our neurofibromatosis clinic we included in a retrospective study all patients with NF1 having at least one peripheral nerve sheath tumour for which they underwent surgery or surgical biopsy. Predictive values for malignant transformation of three clinical symptoms, i.e. pain, enlargement of mass and neurological symptoms, were evaluated in association with histological parameters.
Results Of 69 patients studied, 48 had at least one plexiform neurofibroma and 21 had a malignant peripheral nerve sheath tumour. Only enlargement of the tumour had high negative and positive predictive values for malignant transformation: 0·92 and 0·95, respectively. In multivariate analysis, tumour enlargement was independently associated with malignant transformation (odds ratio 167·8, 95% confidence interval 14·0–2012·1).
Conclusions From a practical point of view, pain, neurological deficit and enlargement of a pre‐existing peripheral nerve sheath tumour in NF1 must lead to deep surgical biopsy to rule out malignant transformation.</abstract><cop>Oxford, UK</cop><pub>Blackwell Science Ltd</pub><pmid>16029330</pmid><doi>10.1111/j.1365-2133.2005.06558.x</doi><tpages>4</tpages></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0007-0963 |
| ispartof | British journal of dermatology (1951), 2005-07, Vol.153 (1), p.79-82 |
| issn | 0007-0963 1365-2133 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_68055810 |
| source | MEDLINE; Oxford University Press Journals All Titles (1996-Current); Wiley Online Library All Journals |
| subjects | Adolescent Adult Aged Biological and medical sciences Disease Progression enlargement Facial Neoplasms - pathology Female Humans Male malignant transformation Medical sciences Middle Aged Nerve Sheath Neoplasms - complications Nerve Sheath Neoplasms - pathology Nervous System Diseases - etiology neurofibromatosis 1 Neurofibromatosis 1 - complications Neurofibromatosis 1 - pathology Neurology Pain - etiology Retrospective Studies Risk Factors Tumors of the nervous system. Phacomatoses |
| title | Symptoms associated with malignancy of peripheral nerve sheath tumours: a retrospective study of 69 patients with neurofibromatosis 1 |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-13T12%3A43%3A54IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_cross&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Symptoms%20associated%20with%20malignancy%20of%20peripheral%20nerve%20sheath%20tumours:%20a%20retrospective%20study%20of%2069%20patients%20with%20neurofibromatosis%201&rft.jtitle=British%20journal%20of%20dermatology%20(1951)&rft.au=Valeyrie-Allanore,%20L.&rft.date=2005-07&rft.volume=153&rft.issue=1&rft.spage=79&rft.epage=82&rft.pages=79-82&rft.issn=0007-0963&rft.eissn=1365-2133&rft.coden=BJDEAZ&rft_id=info:doi/10.1111/j.1365-2133.2005.06558.x&rft_dat=%3Cproquest_cross%3E68055810%3C/proquest_cross%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=200163571&rft_id=info:pmid/16029330&rfr_iscdi=true |