Morphologic and immunophenotypic properties of neoplastic cells in a case of mast cell sarcoma
Mast cell sarcoma is an extremely rare and aggressive type of mast cell disease. Only a few cases have been described so far, and little is known about the biology and phenotype of afflicted cells. We describe morphologic and immunophenotypic properties of neoplastic mast cells in a case of an intra...
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| Veröffentlicht in: | The American journal of surgical pathology 2003-07, Vol.27 (7), p.1013-1019 |
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| container_title | The American journal of surgical pathology |
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| creator | CHOTT, Andreas GUENTHER, Philipp HUEBNER, Angela SELZER, Edgar PARWARESCH, Reza M HORNY, Hans-Peter VALENT, Peter |
| description | Mast cell sarcoma is an extremely rare and aggressive type of mast cell disease. Only a few cases have been described so far, and little is known about the biology and phenotype of afflicted cells. We describe morphologic and immunophenotypic properties of neoplastic mast cells in a case of an intracranial mast cell sarcoma. In Wright-Giemsa-stained cytospin preparations, the morphology of dispersed cells appeared to be highly atypical with a considerable percentage of metachromatic blasts and mast cells with bilobed or multilobed nuclei. Combined toluidine blue/immunofluorescence staining revealed expression of CD13, CD45, CD88, CD116, and CD117 (c-KIT) on neoplastic mast cells. As assessed by immunohistochemistry, mast cells were immunoreactive for tryptase and CD68R, In contrast, the CD2 antigen that is expressed in mast cells in patients with indolent systemic mastocytosis was not detectable. Mast cells also failed to display the c-KIT mutation Asp-816-Val, which is typically found in systemic mast cell disorders. Together, neoplastic mast cells in a case of mast cell sarcoma were found to exhibit unique morphologic, phenotypical, and molecular features when compared with mast cells in indolent mastocytosis or normal tissue mast cells. |
| doi_str_mv | 10.1097/00000478-200307000-00019 |
| format | Article |
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Only a few cases have been described so far, and little is known about the biology and phenotype of afflicted cells. We describe morphologic and immunophenotypic properties of neoplastic mast cells in a case of an intracranial mast cell sarcoma. In Wright-Giemsa-stained cytospin preparations, the morphology of dispersed cells appeared to be highly atypical with a considerable percentage of metachromatic blasts and mast cells with bilobed or multilobed nuclei. Combined toluidine blue/immunofluorescence staining revealed expression of CD13, CD45, CD88, CD116, and CD117 (c-KIT) on neoplastic mast cells. As assessed by immunohistochemistry, mast cells were immunoreactive for tryptase and CD68R, In contrast, the CD2 antigen that is expressed in mast cells in patients with indolent systemic mastocytosis was not detectable. Mast cells also failed to display the c-KIT mutation Asp-816-Val, which is typically found in systemic mast cell disorders. Together, neoplastic mast cells in a case of mast cell sarcoma were found to exhibit unique morphologic, phenotypical, and molecular features when compared with mast cells in indolent mastocytosis or normal tissue mast cells.</description><identifier>ISSN: 0147-5185</identifier><identifier>EISSN: 1532-0979</identifier><identifier>DOI: 10.1097/00000478-200307000-00019</identifier><identifier>PMID: 12826896</identifier><identifier>CODEN: AJSPDX</identifier><language>eng</language><publisher>Hagerstown, MD: Lippincott Williams & Wilkins</publisher><subject>Antigens, CD - metabolism ; Antigens, Surface - metabolism ; Aspartic Acid - genetics ; Biological and medical sciences ; Biomarkers, Tumor - metabolism ; Brain Neoplasms - genetics ; Brain Neoplasms - metabolism ; Brain Neoplasms - pathology ; Cell Nucleus - pathology ; Child ; Female ; Humans ; Immunoenzyme Techniques ; Mast Cells - metabolism ; Mast Cells - pathology ; Mast-Cell Sarcoma - genetics ; Mast-Cell Sarcoma - metabolism ; Mast-Cell Sarcoma - pathology ; Medical sciences ; Neurology ; Phenotype ; Point Mutation ; Proto-Oncogene Proteins c-kit - genetics ; Proto-Oncogene Proteins c-kit - metabolism ; Reverse Transcriptase Polymerase Chain Reaction ; RNA, Messenger - metabolism ; RNA, Neoplasm - analysis ; Serine Endopeptidases - metabolism ; Tryptases ; Tumors of the nervous system. Phacomatoses ; Valine - genetics</subject><ispartof>The American journal of surgical pathology, 2003-07, Vol.27 (7), p.1013-1019</ispartof><rights>2003 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c407t-cc6f501a3e7024cbd1029d29626357d6496b2421e66d5bc8629b0bac600352263</citedby><cites>FETCH-LOGICAL-c407t-cc6f501a3e7024cbd1029d29626357d6496b2421e66d5bc8629b0bac600352263</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27901,27902</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=14971530$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/12826896$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>CHOTT, Andreas</creatorcontrib><creatorcontrib>GUENTHER, Philipp</creatorcontrib><creatorcontrib>HUEBNER, Angela</creatorcontrib><creatorcontrib>SELZER, Edgar</creatorcontrib><creatorcontrib>PARWARESCH, Reza M</creatorcontrib><creatorcontrib>HORNY, Hans-Peter</creatorcontrib><creatorcontrib>VALENT, Peter</creatorcontrib><title>Morphologic and immunophenotypic properties of neoplastic cells in a case of mast cell sarcoma</title><title>The American journal of surgical pathology</title><addtitle>Am J Surg Pathol</addtitle><description>Mast cell sarcoma is an extremely rare and aggressive type of mast cell disease. Only a few cases have been described so far, and little is known about the biology and phenotype of afflicted cells. We describe morphologic and immunophenotypic properties of neoplastic mast cells in a case of an intracranial mast cell sarcoma. In Wright-Giemsa-stained cytospin preparations, the morphology of dispersed cells appeared to be highly atypical with a considerable percentage of metachromatic blasts and mast cells with bilobed or multilobed nuclei. Combined toluidine blue/immunofluorescence staining revealed expression of CD13, CD45, CD88, CD116, and CD117 (c-KIT) on neoplastic mast cells. As assessed by immunohistochemistry, mast cells were immunoreactive for tryptase and CD68R, In contrast, the CD2 antigen that is expressed in mast cells in patients with indolent systemic mastocytosis was not detectable. Mast cells also failed to display the c-KIT mutation Asp-816-Val, which is typically found in systemic mast cell disorders. Together, neoplastic mast cells in a case of mast cell sarcoma were found to exhibit unique morphologic, phenotypical, and molecular features when compared with mast cells in indolent mastocytosis or normal tissue mast cells.</description><subject>Antigens, CD - metabolism</subject><subject>Antigens, Surface - metabolism</subject><subject>Aspartic Acid - genetics</subject><subject>Biological and medical sciences</subject><subject>Biomarkers, Tumor - metabolism</subject><subject>Brain Neoplasms - genetics</subject><subject>Brain Neoplasms - metabolism</subject><subject>Brain Neoplasms - pathology</subject><subject>Cell Nucleus - pathology</subject><subject>Child</subject><subject>Female</subject><subject>Humans</subject><subject>Immunoenzyme Techniques</subject><subject>Mast Cells - metabolism</subject><subject>Mast Cells - pathology</subject><subject>Mast-Cell Sarcoma - genetics</subject><subject>Mast-Cell Sarcoma - metabolism</subject><subject>Mast-Cell Sarcoma - pathology</subject><subject>Medical sciences</subject><subject>Neurology</subject><subject>Phenotype</subject><subject>Point Mutation</subject><subject>Proto-Oncogene Proteins c-kit - genetics</subject><subject>Proto-Oncogene Proteins c-kit - metabolism</subject><subject>Reverse Transcriptase Polymerase Chain Reaction</subject><subject>RNA, Messenger - metabolism</subject><subject>RNA, Neoplasm - analysis</subject><subject>Serine Endopeptidases - metabolism</subject><subject>Tryptases</subject><subject>Tumors of the nervous system. Phacomatoses</subject><subject>Valine - genetics</subject><issn>0147-5185</issn><issn>1532-0979</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2003</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpFkU1PxCAQhonRuOvqXzBc9FYFykc5mo1fyRoverWhlLo1banQHvz3TnerS0ImzPsMzLwghCm5oUSrWzItrrKEEZISBYcENtVHaElFyhJg9DFaEspVImgmFugsxi8gWEbZKVpAZDLTcok-Xnzot77xn7XFpitx3bZj5_ut6_zw00OyD753YahdxL7CnfN9Y-IAgnVNE3HdYYOtiW5SW1B2eRxNsL415-ikMk10F3NcofeH-7f1U7J5fXxe320Sy4kaEmtlJQg1qVOEcVuUlDBdMi2ZTIUqJdeyYJxRJ2UpCptJpgtSGCthfMEAWqHr_b3Q7ffo4pC3dZwaMdDwGHOVchhecACzPWiDjzG4Ku9D3Zrwk1OST97mf97m_97mO2-h9HJ-YyxaVx4KZzMBuJoBE61pqmA6W8cDx7WC3yHpL4fRgRE</recordid><startdate>20030701</startdate><enddate>20030701</enddate><creator>CHOTT, Andreas</creator><creator>GUENTHER, Philipp</creator><creator>HUEBNER, Angela</creator><creator>SELZER, Edgar</creator><creator>PARWARESCH, Reza M</creator><creator>HORNY, Hans-Peter</creator><creator>VALENT, Peter</creator><general>Lippincott Williams & Wilkins</general><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>7X8</scope></search><sort><creationdate>20030701</creationdate><title>Morphologic and immunophenotypic properties of neoplastic cells in a case of mast cell sarcoma</title><author>CHOTT, Andreas ; GUENTHER, Philipp ; HUEBNER, Angela ; SELZER, Edgar ; PARWARESCH, Reza M ; HORNY, Hans-Peter ; VALENT, Peter</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c407t-cc6f501a3e7024cbd1029d29626357d6496b2421e66d5bc8629b0bac600352263</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2003</creationdate><topic>Antigens, CD - metabolism</topic><topic>Antigens, Surface - metabolism</topic><topic>Aspartic Acid - genetics</topic><topic>Biological and medical sciences</topic><topic>Biomarkers, Tumor - metabolism</topic><topic>Brain Neoplasms - genetics</topic><topic>Brain Neoplasms - metabolism</topic><topic>Brain Neoplasms - pathology</topic><topic>Cell Nucleus - pathology</topic><topic>Child</topic><topic>Female</topic><topic>Humans</topic><topic>Immunoenzyme Techniques</topic><topic>Mast Cells - metabolism</topic><topic>Mast Cells - pathology</topic><topic>Mast-Cell Sarcoma - genetics</topic><topic>Mast-Cell Sarcoma - metabolism</topic><topic>Mast-Cell Sarcoma - pathology</topic><topic>Medical sciences</topic><topic>Neurology</topic><topic>Phenotype</topic><topic>Point Mutation</topic><topic>Proto-Oncogene Proteins c-kit - genetics</topic><topic>Proto-Oncogene Proteins c-kit - metabolism</topic><topic>Reverse Transcriptase Polymerase Chain Reaction</topic><topic>RNA, Messenger - metabolism</topic><topic>RNA, Neoplasm - analysis</topic><topic>Serine Endopeptidases - metabolism</topic><topic>Tryptases</topic><topic>Tumors of the nervous system. Phacomatoses</topic><topic>Valine - genetics</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>CHOTT, Andreas</creatorcontrib><creatorcontrib>GUENTHER, Philipp</creatorcontrib><creatorcontrib>HUEBNER, Angela</creatorcontrib><creatorcontrib>SELZER, Edgar</creatorcontrib><creatorcontrib>PARWARESCH, Reza M</creatorcontrib><creatorcontrib>HORNY, Hans-Peter</creatorcontrib><creatorcontrib>VALENT, Peter</creatorcontrib><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>MEDLINE - Academic</collection><jtitle>The American journal of surgical pathology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>CHOTT, Andreas</au><au>GUENTHER, Philipp</au><au>HUEBNER, Angela</au><au>SELZER, Edgar</au><au>PARWARESCH, Reza M</au><au>HORNY, Hans-Peter</au><au>VALENT, Peter</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Morphologic and immunophenotypic properties of neoplastic cells in a case of mast cell sarcoma</atitle><jtitle>The American journal of surgical pathology</jtitle><addtitle>Am J Surg Pathol</addtitle><date>2003-07-01</date><risdate>2003</risdate><volume>27</volume><issue>7</issue><spage>1013</spage><epage>1019</epage><pages>1013-1019</pages><issn>0147-5185</issn><eissn>1532-0979</eissn><coden>AJSPDX</coden><abstract>Mast cell sarcoma is an extremely rare and aggressive type of mast cell disease. Only a few cases have been described so far, and little is known about the biology and phenotype of afflicted cells. We describe morphologic and immunophenotypic properties of neoplastic mast cells in a case of an intracranial mast cell sarcoma. In Wright-Giemsa-stained cytospin preparations, the morphology of dispersed cells appeared to be highly atypical with a considerable percentage of metachromatic blasts and mast cells with bilobed or multilobed nuclei. Combined toluidine blue/immunofluorescence staining revealed expression of CD13, CD45, CD88, CD116, and CD117 (c-KIT) on neoplastic mast cells. As assessed by immunohistochemistry, mast cells were immunoreactive for tryptase and CD68R, In contrast, the CD2 antigen that is expressed in mast cells in patients with indolent systemic mastocytosis was not detectable. Mast cells also failed to display the c-KIT mutation Asp-816-Val, which is typically found in systemic mast cell disorders. Together, neoplastic mast cells in a case of mast cell sarcoma were found to exhibit unique morphologic, phenotypical, and molecular features when compared with mast cells in indolent mastocytosis or normal tissue mast cells.</abstract><cop>Hagerstown, MD</cop><pub>Lippincott Williams & Wilkins</pub><pmid>12826896</pmid><doi>10.1097/00000478-200307000-00019</doi><tpages>7</tpages></addata></record> |
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| subjects | Antigens, CD - metabolism Antigens, Surface - metabolism Aspartic Acid - genetics Biological and medical sciences Biomarkers, Tumor - metabolism Brain Neoplasms - genetics Brain Neoplasms - metabolism Brain Neoplasms - pathology Cell Nucleus - pathology Child Female Humans Immunoenzyme Techniques Mast Cells - metabolism Mast Cells - pathology Mast-Cell Sarcoma - genetics Mast-Cell Sarcoma - metabolism Mast-Cell Sarcoma - pathology Medical sciences Neurology Phenotype Point Mutation Proto-Oncogene Proteins c-kit - genetics Proto-Oncogene Proteins c-kit - metabolism Reverse Transcriptase Polymerase Chain Reaction RNA, Messenger - metabolism RNA, Neoplasm - analysis Serine Endopeptidases - metabolism Tryptases Tumors of the nervous system. Phacomatoses Valine - genetics |
| title | Morphologic and immunophenotypic properties of neoplastic cells in a case of mast cell sarcoma |
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