Primary Vulvar and Vaginal Extraosseous Ewing's Sarcoma/Peripheral Neuroectodermal Tumor: Diagnostic Confirmation with CD99 Immunostaining and Reverse Transcriptase-Polymerase Chain Reaction
Two cases of extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor arising in unusual, superficial sites are reported. One tumor involved the vaginal wall of a 35-year-old woman, and the other neoplasm arose in the dermis of the vulva in a 28-year-old woman. The tumors showed characteri...
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Veröffentlicht in: | International journal of gynecological pathology 2000-04, Vol.19 (2), p.103-109 |
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description | Two cases of extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor arising in unusual, superficial sites are reported. One tumor involved the vaginal wall of a 35-year-old woman, and the other neoplasm arose in the dermis of the vulva in a 28-year-old woman. The tumors showed characteristic microscopic features of Ewing's sarcoma/peripheral neuroectodermal tumor with nodular monotonous proliferations of undifferentiated, small, round, hyperchromatic cells with a low mitotic index. Rare rosette-like formations were apparent only in the vulvar neoplasm. The tumors displayed intense immunoreactivity in a membranous pattern for CD99, the cell surface glycoprotein encoded by the MIC2 gene. Genetically, the tumors expressed the EWS/FLI-1 chimeric transcript, derived from the t(11;22)(q24;q12) chromosomal translocation. Both patients had localized disease treated with wide local excision; one received postoperative chemotherapy, and the other received chemotherapy and radiotherapy. To date, 18 and 19 months after diagnosis, neither patient has had clinical evidence of local recurrence or metastasis. To our knowledge, these are the first reported cases of vaginal and vulvar Ewing's sarcoma/peripheral neuroectodermal tumor, confirmed with molecular genetic analysis, in the English literature. |
doi_str_mv | 10.1097/00004347-200004000-00002 |
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One tumor involved the vaginal wall of a 35-year-old woman, and the other neoplasm arose in the dermis of the vulva in a 28-year-old woman. The tumors showed characteristic microscopic features of Ewing's sarcoma/peripheral neuroectodermal tumor with nodular monotonous proliferations of undifferentiated, small, round, hyperchromatic cells with a low mitotic index. Rare rosette-like formations were apparent only in the vulvar neoplasm. The tumors displayed intense immunoreactivity in a membranous pattern for CD99, the cell surface glycoprotein encoded by the MIC2 gene. Genetically, the tumors expressed the EWS/FLI-1 chimeric transcript, derived from the t(11;22)(q24;q12) chromosomal translocation. Both patients had localized disease treated with wide local excision; one received postoperative chemotherapy, and the other received chemotherapy and radiotherapy. To date, 18 and 19 months after diagnosis, neither patient has had clinical evidence of local recurrence or metastasis. To our knowledge, these are the first reported cases of vaginal and vulvar Ewing's sarcoma/peripheral neuroectodermal tumor, confirmed with molecular genetic analysis, in the English literature.</description><identifier>ISSN: 0277-1691</identifier><identifier>EISSN: 1538-7151</identifier><identifier>DOI: 10.1097/00004347-200004000-00002</identifier><identifier>PMID: 10782405</identifier><identifier>CODEN: IJGPDR</identifier><language>eng</language><publisher>Philadelphia, PA: Lippincott Williams & Wilkins, Inc</publisher><subject>12E7 Antigen ; Adult ; Antigens, CD - analysis ; Antineoplastic Agents - therapeutic use ; Biological and medical sciences ; Cell Adhesion Molecules - analysis ; Chromosomes, Human, Pair 11 ; Chromosomes, Human, Pair 22 ; Female ; Genital system. Mammary gland ; Humans ; Immunohistochemistry ; Investigative techniques, diagnostic techniques (general aspects) ; Medical sciences ; Neuroectodermal Tumors - diagnosis ; Neuroectodermal Tumors - genetics ; Neuroectodermal Tumors - therapy ; Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques ; Radiotherapy ; Reverse Transcriptase Polymerase Chain Reaction ; Sarcoma, Ewing - diagnosis ; Sarcoma, Ewing - genetics ; Sarcoma, Ewing - therapy ; Surgical Procedures, Operative ; Translocation, Genetic ; Vaginal Neoplasms - diagnosis ; Vaginal Neoplasms - genetics ; Vaginal Neoplasms - therapy ; Vulvar Neoplasms - diagnosis ; Vulvar Neoplasms - genetics ; Vulvar Neoplasms - therapy</subject><ispartof>International journal of gynecological pathology, 2000-04, Vol.19 (2), p.103-109</ispartof><rights>2000 Lippincott Williams & Wilkins, Inc.</rights><rights>2000 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3852-31f2382e2362202f3d22b84bfc12129a551bfdcdc6f22af016cb93bee400a2443</citedby><cites>FETCH-LOGICAL-c3852-31f2382e2362202f3d22b84bfc12129a551bfdcdc6f22af016cb93bee400a2443</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27923,27924</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=1328157$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/10782405$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Vang, Russell</creatorcontrib><creatorcontrib>Taubenberger, Jeffery K</creatorcontrib><creatorcontrib>Mannion, Ciaran M</creatorcontrib><creatorcontrib>Bijwaard, Karen</creatorcontrib><creatorcontrib>Malpica, Anais</creatorcontrib><creatorcontrib>Ordonez, Nelson G</creatorcontrib><creatorcontrib>Tavassoli, Fattaneh A</creatorcontrib><creatorcontrib>Silver, Susan A</creatorcontrib><title>Primary Vulvar and Vaginal Extraosseous Ewing's Sarcoma/Peripheral Neuroectodermal Tumor: Diagnostic Confirmation with CD99 Immunostaining and Reverse Transcriptase-Polymerase Chain Reaction</title><title>International journal of gynecological pathology</title><addtitle>Int J Gynecol Pathol</addtitle><description>Two cases of extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor arising in unusual, superficial sites are reported. One tumor involved the vaginal wall of a 35-year-old woman, and the other neoplasm arose in the dermis of the vulva in a 28-year-old woman. The tumors showed characteristic microscopic features of Ewing's sarcoma/peripheral neuroectodermal tumor with nodular monotonous proliferations of undifferentiated, small, round, hyperchromatic cells with a low mitotic index. Rare rosette-like formations were apparent only in the vulvar neoplasm. The tumors displayed intense immunoreactivity in a membranous pattern for CD99, the cell surface glycoprotein encoded by the MIC2 gene. Genetically, the tumors expressed the EWS/FLI-1 chimeric transcript, derived from the t(11;22)(q24;q12) chromosomal translocation. Both patients had localized disease treated with wide local excision; one received postoperative chemotherapy, and the other received chemotherapy and radiotherapy. To date, 18 and 19 months after diagnosis, neither patient has had clinical evidence of local recurrence or metastasis. To our knowledge, these are the first reported cases of vaginal and vulvar Ewing's sarcoma/peripheral neuroectodermal tumor, confirmed with molecular genetic analysis, in the English literature.</description><subject>12E7 Antigen</subject><subject>Adult</subject><subject>Antigens, CD - analysis</subject><subject>Antineoplastic Agents - therapeutic use</subject><subject>Biological and medical sciences</subject><subject>Cell Adhesion Molecules - analysis</subject><subject>Chromosomes, Human, Pair 11</subject><subject>Chromosomes, Human, Pair 22</subject><subject>Female</subject><subject>Genital system. Mammary gland</subject><subject>Humans</subject><subject>Immunohistochemistry</subject><subject>Investigative techniques, diagnostic techniques (general aspects)</subject><subject>Medical sciences</subject><subject>Neuroectodermal Tumors - diagnosis</subject><subject>Neuroectodermal Tumors - genetics</subject><subject>Neuroectodermal Tumors - therapy</subject><subject>Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques</subject><subject>Radiotherapy</subject><subject>Reverse Transcriptase Polymerase Chain Reaction</subject><subject>Sarcoma, Ewing - diagnosis</subject><subject>Sarcoma, Ewing - genetics</subject><subject>Sarcoma, Ewing - therapy</subject><subject>Surgical Procedures, Operative</subject><subject>Translocation, Genetic</subject><subject>Vaginal Neoplasms - diagnosis</subject><subject>Vaginal Neoplasms - genetics</subject><subject>Vaginal Neoplasms - therapy</subject><subject>Vulvar Neoplasms - diagnosis</subject><subject>Vulvar Neoplasms - genetics</subject><subject>Vulvar Neoplasms - therapy</subject><issn>0277-1691</issn><issn>1538-7151</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2000</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp1kttu1DAQhi0EokvhFZAvEFyF2uMcuUPpApUqWMHS22ji2BtDEm_tpEtfjmfD2SyHGyxZHnu-mV_6x4RQzl5zVmQXLKxYxFkExyjsaA7gAVnxRORRxhP-kKwYZFnE04KfkSfef2OMpzzNHpMzzrIcYpasyM-NMz26e3ozdXfoKA4NvcGdGbCj6x-jQ-u9spOn64MZdq88_YJO2h4vNsqZfatc4D6qyVklR9so14f7duqte0MvDe4G60cjaWkHbUJuNHagBzO2tLwsCnrV99NMoBlC86P2Z3WnnFd063DwMkiM6FW0sd19H7RComwDHTCUc7On5JHGzqtnp_OcfH233pYfoutP76_Kt9eRFHkCkeAaRA4KRArAQIsGoM7jWksOHApMEl7rRjYy1QCog0-yLkStVLAWIY7FOXm59N07ezspP1a98VJ1HQ6zO1XGWZpDmgcwX0DpgnNO6Wq_GFxxVs2zq37Prvozu-MThNLnJ42p7lXzT-EyrAC8OAHoJXY6WCSN_8sJyHmSBSxesIPtxmDm9246KFe1Cruxrf73dcQv5l60tQ</recordid><startdate>200004</startdate><enddate>200004</enddate><creator>Vang, Russell</creator><creator>Taubenberger, Jeffery K</creator><creator>Mannion, Ciaran M</creator><creator>Bijwaard, Karen</creator><creator>Malpica, Anais</creator><creator>Ordonez, Nelson G</creator><creator>Tavassoli, Fattaneh A</creator><creator>Silver, Susan A</creator><general>Lippincott Williams & Wilkins, Inc</general><general>Lippincott</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>200004</creationdate><title>Primary Vulvar and Vaginal Extraosseous Ewing's Sarcoma/Peripheral Neuroectodermal Tumor: Diagnostic Confirmation with CD99 Immunostaining and Reverse Transcriptase-Polymerase Chain Reaction</title><author>Vang, Russell ; Taubenberger, Jeffery K ; Mannion, Ciaran M ; Bijwaard, Karen ; Malpica, Anais ; Ordonez, Nelson G ; Tavassoli, Fattaneh A ; Silver, Susan A</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3852-31f2382e2362202f3d22b84bfc12129a551bfdcdc6f22af016cb93bee400a2443</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2000</creationdate><topic>12E7 Antigen</topic><topic>Adult</topic><topic>Antigens, CD - analysis</topic><topic>Antineoplastic Agents - therapeutic use</topic><topic>Biological and medical sciences</topic><topic>Cell Adhesion Molecules - analysis</topic><topic>Chromosomes, Human, Pair 11</topic><topic>Chromosomes, Human, Pair 22</topic><topic>Female</topic><topic>Genital system. Mammary gland</topic><topic>Humans</topic><topic>Immunohistochemistry</topic><topic>Investigative techniques, diagnostic techniques (general aspects)</topic><topic>Medical sciences</topic><topic>Neuroectodermal Tumors - diagnosis</topic><topic>Neuroectodermal Tumors - genetics</topic><topic>Neuroectodermal Tumors - therapy</topic><topic>Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques</topic><topic>Radiotherapy</topic><topic>Reverse Transcriptase Polymerase Chain Reaction</topic><topic>Sarcoma, Ewing - diagnosis</topic><topic>Sarcoma, Ewing - genetics</topic><topic>Sarcoma, Ewing - therapy</topic><topic>Surgical Procedures, Operative</topic><topic>Translocation, Genetic</topic><topic>Vaginal Neoplasms - diagnosis</topic><topic>Vaginal Neoplasms - genetics</topic><topic>Vaginal Neoplasms - therapy</topic><topic>Vulvar Neoplasms - diagnosis</topic><topic>Vulvar Neoplasms - genetics</topic><topic>Vulvar Neoplasms - therapy</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Vang, Russell</creatorcontrib><creatorcontrib>Taubenberger, Jeffery K</creatorcontrib><creatorcontrib>Mannion, Ciaran M</creatorcontrib><creatorcontrib>Bijwaard, Karen</creatorcontrib><creatorcontrib>Malpica, Anais</creatorcontrib><creatorcontrib>Ordonez, Nelson G</creatorcontrib><creatorcontrib>Tavassoli, Fattaneh A</creatorcontrib><creatorcontrib>Silver, Susan A</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>International journal of gynecological pathology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Vang, Russell</au><au>Taubenberger, Jeffery K</au><au>Mannion, Ciaran M</au><au>Bijwaard, Karen</au><au>Malpica, Anais</au><au>Ordonez, Nelson G</au><au>Tavassoli, Fattaneh A</au><au>Silver, Susan A</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Primary Vulvar and Vaginal Extraosseous Ewing's Sarcoma/Peripheral Neuroectodermal Tumor: Diagnostic Confirmation with CD99 Immunostaining and Reverse Transcriptase-Polymerase Chain Reaction</atitle><jtitle>International journal of gynecological pathology</jtitle><addtitle>Int J Gynecol Pathol</addtitle><date>2000-04</date><risdate>2000</risdate><volume>19</volume><issue>2</issue><spage>103</spage><epage>109</epage><pages>103-109</pages><issn>0277-1691</issn><eissn>1538-7151</eissn><coden>IJGPDR</coden><abstract>Two cases of extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor arising in unusual, superficial sites are reported. One tumor involved the vaginal wall of a 35-year-old woman, and the other neoplasm arose in the dermis of the vulva in a 28-year-old woman. The tumors showed characteristic microscopic features of Ewing's sarcoma/peripheral neuroectodermal tumor with nodular monotonous proliferations of undifferentiated, small, round, hyperchromatic cells with a low mitotic index. Rare rosette-like formations were apparent only in the vulvar neoplasm. The tumors displayed intense immunoreactivity in a membranous pattern for CD99, the cell surface glycoprotein encoded by the MIC2 gene. Genetically, the tumors expressed the EWS/FLI-1 chimeric transcript, derived from the t(11;22)(q24;q12) chromosomal translocation. Both patients had localized disease treated with wide local excision; one received postoperative chemotherapy, and the other received chemotherapy and radiotherapy. To date, 18 and 19 months after diagnosis, neither patient has had clinical evidence of local recurrence or metastasis. To our knowledge, these are the first reported cases of vaginal and vulvar Ewing's sarcoma/peripheral neuroectodermal tumor, confirmed with molecular genetic analysis, in the English literature.</abstract><cop>Philadelphia, PA</cop><cop>Hagerstown, MD</cop><pub>Lippincott Williams & Wilkins, Inc</pub><pmid>10782405</pmid><doi>10.1097/00004347-200004000-00002</doi><tpages>7</tpages></addata></record> |
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subjects | 12E7 Antigen Adult Antigens, CD - analysis Antineoplastic Agents - therapeutic use Biological and medical sciences Cell Adhesion Molecules - analysis Chromosomes, Human, Pair 11 Chromosomes, Human, Pair 22 Female Genital system. Mammary gland Humans Immunohistochemistry Investigative techniques, diagnostic techniques (general aspects) Medical sciences Neuroectodermal Tumors - diagnosis Neuroectodermal Tumors - genetics Neuroectodermal Tumors - therapy Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques Radiotherapy Reverse Transcriptase Polymerase Chain Reaction Sarcoma, Ewing - diagnosis Sarcoma, Ewing - genetics Sarcoma, Ewing - therapy Surgical Procedures, Operative Translocation, Genetic Vaginal Neoplasms - diagnosis Vaginal Neoplasms - genetics Vaginal Neoplasms - therapy Vulvar Neoplasms - diagnosis Vulvar Neoplasms - genetics Vulvar Neoplasms - therapy |
title | Primary Vulvar and Vaginal Extraosseous Ewing's Sarcoma/Peripheral Neuroectodermal Tumor: Diagnostic Confirmation with CD99 Immunostaining and Reverse Transcriptase-Polymerase Chain Reaction |
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