Low grade malignant peripheral nerve sheath tumor with smooth muscle differentiation
Malignant peripheral nerve sheath tumors are usually Schwann cell derived. Differentiation along mesenchymal lines is uncommon. Herein we present an example with smooth muscle differentiation occurring in the left distal forearm of a 62-year-old male with neurofibromatosis type I. Incisional biopsy...
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Veröffentlicht in: | Acta neuropathologica 2007-06, Vol.113 (6), p.705-709 |
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description | Malignant peripheral nerve sheath tumors are usually Schwann cell derived. Differentiation along mesenchymal lines is uncommon. Herein we present an example with smooth muscle differentiation occurring in the left distal forearm of a 62-year-old male with neurofibromatosis type I. Incisional biopsy of the slowly growing mass demonstrated a low-grade malignant peripheral nerve sheath tumor with strong S-100 protein immunoexpression as well as focal smooth muscle actin staining in atypical neoplastic cells. Immunostains for epithelial membrane antigen, neurofilament protein, glial fibrillary acidic protein, CD57, desmin, and myogenin were negative. The MIB-1 labeling index was 5.4%. Electron microscopy revealed arrays of microfilaments (actin myofilaments) variably associated with fusiform dense bodies, plasmalemmal micropinocytotic vesicles, and foci of external lamina (basement membrane) consistent with smooth muscle differentiation. Smooth muscle should be added to the spectrum of differentiation exhibited by malignant peripheral nerve sheath tumors. Its recognition requires exclusion of the alternative diagnosis of leiomyosarcoma. |
doi_str_mv | 10.1007/s00401-006-0171-8 |
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Differentiation along mesenchymal lines is uncommon. Herein we present an example with smooth muscle differentiation occurring in the left distal forearm of a 62-year-old male with neurofibromatosis type I. Incisional biopsy of the slowly growing mass demonstrated a low-grade malignant peripheral nerve sheath tumor with strong S-100 protein immunoexpression as well as focal smooth muscle actin staining in atypical neoplastic cells. Immunostains for epithelial membrane antigen, neurofilament protein, glial fibrillary acidic protein, CD57, desmin, and myogenin were negative. The MIB-1 labeling index was 5.4%. Electron microscopy revealed arrays of microfilaments (actin myofilaments) variably associated with fusiform dense bodies, plasmalemmal micropinocytotic vesicles, and foci of external lamina (basement membrane) consistent with smooth muscle differentiation. Smooth muscle should be added to the spectrum of differentiation exhibited by malignant peripheral nerve sheath tumors. Its recognition requires exclusion of the alternative diagnosis of leiomyosarcoma.</description><identifier>ISSN: 0001-6322</identifier><identifier>EISSN: 1432-0533</identifier><identifier>DOI: 10.1007/s00401-006-0171-8</identifier><identifier>PMID: 17119986</identifier><language>eng</language><publisher>Germany: Springer Nature B.V</publisher><subject>2-Methylisoborneol ; Actin ; Biopsy ; Bone Neoplasms - secondary ; CD57 antigen ; Cell differentiation ; Cell Differentiation - physiology ; Desmin ; Electron microscopy ; Fatal Outcome ; Forearm ; Forearm - pathology ; Genetic disorders ; Glial fibrillary acidic protein ; Humans ; Immunohistochemistry ; Low Back Pain - etiology ; Male ; Membrane proteins ; Mesenchyme ; Microfilaments ; Microscopy, Electron ; Middle Aged ; Muscle, Smooth - pathology ; Myogenin ; Nerve Sheath Neoplasms - pathology ; Neurofibromatosis ; Neurofibromatosis 1 - complications ; Neurofibromatosis 1 - pathology ; Neurofilaments ; Neurological disorders ; Neuronal-glial interactions ; Paraffin Embedding ; Peripheral nerves ; Proteins ; S100 protein ; S100 Proteins - metabolism ; Skin Neoplasms - pathology ; Smooth muscle ; Tumors</subject><ispartof>Acta neuropathologica, 2007-06, Vol.113 (6), p.705-709</ispartof><rights>Springer-Verlag 2007</rights><rights>Springer-Verlag 2006.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c386t-d0fd3db0e35846645c8495563d2412b72e49386c11e0ec0e7ad5472541798edb3</citedby><cites>FETCH-LOGICAL-c386t-d0fd3db0e35846645c8495563d2412b72e49386c11e0ec0e7ad5472541798edb3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27903,27904</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/17119986$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Rodriguez, Fausto J</creatorcontrib><creatorcontrib>Scheithauer, Bernd W</creatorcontrib><creatorcontrib>Abell-Aleff, Patrice C</creatorcontrib><creatorcontrib>Elamin, Elshami</creatorcontrib><creatorcontrib>Erlandson, Robert A</creatorcontrib><title>Low grade malignant peripheral nerve sheath tumor with smooth muscle differentiation</title><title>Acta neuropathologica</title><addtitle>Acta Neuropathol</addtitle><description>Malignant peripheral nerve sheath tumors are usually Schwann cell derived. Differentiation along mesenchymal lines is uncommon. Herein we present an example with smooth muscle differentiation occurring in the left distal forearm of a 62-year-old male with neurofibromatosis type I. Incisional biopsy of the slowly growing mass demonstrated a low-grade malignant peripheral nerve sheath tumor with strong S-100 protein immunoexpression as well as focal smooth muscle actin staining in atypical neoplastic cells. Immunostains for epithelial membrane antigen, neurofilament protein, glial fibrillary acidic protein, CD57, desmin, and myogenin were negative. The MIB-1 labeling index was 5.4%. Electron microscopy revealed arrays of microfilaments (actin myofilaments) variably associated with fusiform dense bodies, plasmalemmal micropinocytotic vesicles, and foci of external lamina (basement membrane) consistent with smooth muscle differentiation. Smooth muscle should be added to the spectrum of differentiation exhibited by malignant peripheral nerve sheath tumors. Its recognition requires exclusion of the alternative diagnosis of leiomyosarcoma.</description><subject>2-Methylisoborneol</subject><subject>Actin</subject><subject>Biopsy</subject><subject>Bone Neoplasms - secondary</subject><subject>CD57 antigen</subject><subject>Cell differentiation</subject><subject>Cell Differentiation - physiology</subject><subject>Desmin</subject><subject>Electron microscopy</subject><subject>Fatal Outcome</subject><subject>Forearm</subject><subject>Forearm - pathology</subject><subject>Genetic disorders</subject><subject>Glial fibrillary acidic protein</subject><subject>Humans</subject><subject>Immunohistochemistry</subject><subject>Low Back Pain - etiology</subject><subject>Male</subject><subject>Membrane proteins</subject><subject>Mesenchyme</subject><subject>Microfilaments</subject><subject>Microscopy, Electron</subject><subject>Middle Aged</subject><subject>Muscle, Smooth - pathology</subject><subject>Myogenin</subject><subject>Nerve Sheath Neoplasms - pathology</subject><subject>Neurofibromatosis</subject><subject>Neurofibromatosis 1 - complications</subject><subject>Neurofibromatosis 1 - pathology</subject><subject>Neurofilaments</subject><subject>Neurological disorders</subject><subject>Neuronal-glial interactions</subject><subject>Paraffin Embedding</subject><subject>Peripheral nerves</subject><subject>Proteins</subject><subject>S100 protein</subject><subject>S100 Proteins - metabolism</subject><subject>Skin Neoplasms - pathology</subject><subject>Smooth muscle</subject><subject>Tumors</subject><issn>0001-6322</issn><issn>1432-0533</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2007</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><recordid>eNp90U1LxDAQBuAgiq6rP8CLFAU9VSff7VHEL1jwoueQbaZupW3WpHXx35tlFwRBT5OQZwYmLyEnFK4ogL6OAAJoDqByoJrmxQ6ZUMFZDpLzXTIBSK-KM3ZADmN8TzemhdwnBwnTsizUhLzM_Cp7C9Zh1tm2eettP2RLDM1ygcG2WY_hE7O4QDsssmHsfMhWTTrGzvtUujFWLWauqWsM2A-NHRrfH5G92rYRj7d1Sl7v715uH_PZ88PT7c0sr3ihhtxB7bibA3JZCKWErApRSqm4Y4KyuWYoygQrShGwAtTWSaGZFFSXBbo5n5LLzdxl8B8jxsF0TaywbW2PfoxGc8Gg5FomefGvpKUGWSqR4Pkv-O7H0KctDFNKcp2-jSd19qeitFC8SGpK6AZVwccYsDbL0HQ2fBkKZp2f2eRnUn5mnZ9Z95xuB4_zDt1PxzYw_g3Hu5QS</recordid><startdate>200706</startdate><enddate>200706</enddate><creator>Rodriguez, Fausto J</creator><creator>Scheithauer, Bernd W</creator><creator>Abell-Aleff, Patrice C</creator><creator>Elamin, Elshami</creator><creator>Erlandson, Robert A</creator><general>Springer Nature B.V</general><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>3V.</scope><scope>7TK</scope><scope>7U9</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>88G</scope><scope>8AO</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>H94</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>M2M</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>PSYQQ</scope><scope>Q9U</scope><scope>7X8</scope></search><sort><creationdate>200706</creationdate><title>Low grade malignant peripheral nerve sheath tumor with smooth muscle differentiation</title><author>Rodriguez, Fausto J ; Scheithauer, Bernd W ; Abell-Aleff, Patrice C ; Elamin, Elshami ; Erlandson, Robert A</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c386t-d0fd3db0e35846645c8495563d2412b72e49386c11e0ec0e7ad5472541798edb3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2007</creationdate><topic>2-Methylisoborneol</topic><topic>Actin</topic><topic>Biopsy</topic><topic>Bone Neoplasms - secondary</topic><topic>CD57 antigen</topic><topic>Cell differentiation</topic><topic>Cell Differentiation - physiology</topic><topic>Desmin</topic><topic>Electron microscopy</topic><topic>Fatal Outcome</topic><topic>Forearm</topic><topic>Forearm - pathology</topic><topic>Genetic disorders</topic><topic>Glial fibrillary acidic protein</topic><topic>Humans</topic><topic>Immunohistochemistry</topic><topic>Low Back Pain - etiology</topic><topic>Male</topic><topic>Membrane proteins</topic><topic>Mesenchyme</topic><topic>Microfilaments</topic><topic>Microscopy, Electron</topic><topic>Middle Aged</topic><topic>Muscle, Smooth - pathology</topic><topic>Myogenin</topic><topic>Nerve Sheath Neoplasms - pathology</topic><topic>Neurofibromatosis</topic><topic>Neurofibromatosis 1 - complications</topic><topic>Neurofibromatosis 1 - pathology</topic><topic>Neurofilaments</topic><topic>Neurological disorders</topic><topic>Neuronal-glial interactions</topic><topic>Paraffin Embedding</topic><topic>Peripheral nerves</topic><topic>Proteins</topic><topic>S100 protein</topic><topic>S100 Proteins - metabolism</topic><topic>Skin Neoplasms - pathology</topic><topic>Smooth muscle</topic><topic>Tumors</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Rodriguez, Fausto J</creatorcontrib><creatorcontrib>Scheithauer, Bernd W</creatorcontrib><creatorcontrib>Abell-Aleff, Patrice C</creatorcontrib><creatorcontrib>Elamin, Elshami</creatorcontrib><creatorcontrib>Erlandson, Robert A</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Neurosciences Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>Psychology Database (Alumni)</collection><collection>ProQuest Pharma Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central Essentials</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>ProQuest Psychology</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>ProQuest One Psychology</collection><collection>ProQuest Central Basic</collection><collection>MEDLINE - Academic</collection><jtitle>Acta neuropathologica</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Rodriguez, Fausto J</au><au>Scheithauer, Bernd W</au><au>Abell-Aleff, Patrice C</au><au>Elamin, Elshami</au><au>Erlandson, Robert A</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Low grade malignant peripheral nerve sheath tumor with smooth muscle differentiation</atitle><jtitle>Acta neuropathologica</jtitle><addtitle>Acta Neuropathol</addtitle><date>2007-06</date><risdate>2007</risdate><volume>113</volume><issue>6</issue><spage>705</spage><epage>709</epage><pages>705-709</pages><issn>0001-6322</issn><eissn>1432-0533</eissn><abstract>Malignant peripheral nerve sheath tumors are usually Schwann cell derived. Differentiation along mesenchymal lines is uncommon. Herein we present an example with smooth muscle differentiation occurring in the left distal forearm of a 62-year-old male with neurofibromatosis type I. Incisional biopsy of the slowly growing mass demonstrated a low-grade malignant peripheral nerve sheath tumor with strong S-100 protein immunoexpression as well as focal smooth muscle actin staining in atypical neoplastic cells. Immunostains for epithelial membrane antigen, neurofilament protein, glial fibrillary acidic protein, CD57, desmin, and myogenin were negative. The MIB-1 labeling index was 5.4%. Electron microscopy revealed arrays of microfilaments (actin myofilaments) variably associated with fusiform dense bodies, plasmalemmal micropinocytotic vesicles, and foci of external lamina (basement membrane) consistent with smooth muscle differentiation. Smooth muscle should be added to the spectrum of differentiation exhibited by malignant peripheral nerve sheath tumors. Its recognition requires exclusion of the alternative diagnosis of leiomyosarcoma.</abstract><cop>Germany</cop><pub>Springer Nature B.V</pub><pmid>17119986</pmid><doi>10.1007/s00401-006-0171-8</doi><tpages>5</tpages></addata></record> |
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subjects | 2-Methylisoborneol Actin Biopsy Bone Neoplasms - secondary CD57 antigen Cell differentiation Cell Differentiation - physiology Desmin Electron microscopy Fatal Outcome Forearm Forearm - pathology Genetic disorders Glial fibrillary acidic protein Humans Immunohistochemistry Low Back Pain - etiology Male Membrane proteins Mesenchyme Microfilaments Microscopy, Electron Middle Aged Muscle, Smooth - pathology Myogenin Nerve Sheath Neoplasms - pathology Neurofibromatosis Neurofibromatosis 1 - complications Neurofibromatosis 1 - pathology Neurofilaments Neurological disorders Neuronal-glial interactions Paraffin Embedding Peripheral nerves Proteins S100 protein S100 Proteins - metabolism Skin Neoplasms - pathology Smooth muscle Tumors |
title | Low grade malignant peripheral nerve sheath tumor with smooth muscle differentiation |
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