Recurrent vulvar melanoma in a patient with neurofibromatosis and gastrointestinal stromal tumour
We report a case of a 51-year-old woman with neurofibromatosis who presented in 2012 with postmenopausal bleeding. Excision biopsy of a pigmented lesion of the labia minora was consistent with an ulcerated vulvar BRAF wild type malignant melanoma (MM). Initial excision was followed by radical vulvec...
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description | We report a case of a 51-year-old woman with neurofibromatosis who presented in 2012 with postmenopausal bleeding. Excision biopsy of a pigmented lesion of the labia minora was consistent with an ulcerated vulvar BRAF wild type malignant melanoma (MM). Initial excision was followed by radical vulvectomy and adjuvant interferon. Local recurrence in January 2017 was further resected. Positron emission tomography (PET)-CT in May 2017 identified an FDG avid omental deposit; consistent histologically with MM when resected. Postoperative PET-CT in August 2017 demonstrated local recurrence. In the setting of resected stage IV disease and a third local recurrence, the decision was made to instigate immunotherapy. Vulvar melanoma is rare accounting for 0.2% of all melanoma. Presentation is typically a decade later than cutaneous melanoma with a tendency to late metastases and poorer prognosis. Given their rarity the treatment paradigm is less clearly defined and largely extrapolated from that of cutaneous melanomas. |
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Excision biopsy of a pigmented lesion of the labia minora was consistent with an ulcerated vulvar BRAF wild type malignant melanoma (MM). Initial excision was followed by radical vulvectomy and adjuvant interferon. Local recurrence in January 2017 was further resected. Positron emission tomography (PET)-CT in May 2017 identified an FDG avid omental deposit; consistent histologically with MM when resected. Postoperative PET-CT in August 2017 demonstrated local recurrence. In the setting of resected stage IV disease and a third local recurrence, the decision was made to instigate immunotherapy. Vulvar melanoma is rare accounting for 0.2% of all melanoma. Presentation is typically a decade later than cutaneous melanoma with a tendency to late metastases and poorer prognosis. Given their rarity the treatment paradigm is less clearly defined and largely extrapolated from that of cutaneous melanomas.</description><identifier>ISSN: 1757-790X</identifier><identifier>EISSN: 1757-790X</identifier><identifier>DOI: 10.1136/bcr-2018-224744</identifier><identifier>PMID: 30665926</identifier><language>eng</language><publisher>England: BMJ Publishing Group LTD</publisher><subject>Biopsy ; Case reports ; Colorectal cancer ; Dermatology ; Female ; Gastrointestinal cancer ; Gastrointestinal Stromal Tumors - diagnostic imaging ; Gastrointestinal Stromal Tumors - drug therapy ; Genetic disorders ; Humans ; Immunotherapy ; Medical imaging ; Melanoma ; Melanoma - diagnostic imaging ; Melanoma - drug therapy ; Middle Aged ; Mutation ; Neoplasm Recurrence, Local - diagnostic imaging ; Neoplasm Recurrence, Local - drug therapy ; Neurofibromatoses - diagnostic imaging ; Neurofibromatoses - drug therapy ; Neurological disorders ; Positron Emission Tomography Computed Tomography ; Rare Disease ; Skin cancer ; Treatment Outcome ; Tumors ; Vulvar Neoplasms - diagnostic imaging ; Vulvar Neoplasms - drug therapy</subject><ispartof>BMJ case reports, 2019-01, Vol.12 (1), p.e224744</ispartof><rights>BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.</rights><rights>2019 BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.</rights><rights>BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ. 2019</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-b410t-c9f882d73929bb913b780b75682ab41907e94b042288f2ca0eac2143cc9c9e483</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC6340566/pdf/$$EPDF$$P50$$Gpubmedcentral$$H</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC6340566/$$EHTML$$P50$$Gpubmedcentral$$H</linktohtml><link.rule.ids>230,314,723,776,780,881,27901,27902,53766,53768</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/30665926$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Linehan, Anna</creatorcontrib><creatorcontrib>Harrold, Emily</creatorcontrib><creatorcontrib>Pilson, Keith</creatorcontrib><creatorcontrib>McCaffrey, John</creatorcontrib><title>Recurrent vulvar melanoma in a patient with neurofibromatosis and gastrointestinal stromal tumour</title><title>BMJ case reports</title><addtitle>BMJ Case Rep</addtitle><description>We report a case of a 51-year-old woman with neurofibromatosis who presented in 2012 with postmenopausal bleeding. Excision biopsy of a pigmented lesion of the labia minora was consistent with an ulcerated vulvar BRAF wild type malignant melanoma (MM). Initial excision was followed by radical vulvectomy and adjuvant interferon. Local recurrence in January 2017 was further resected. Positron emission tomography (PET)-CT in May 2017 identified an FDG avid omental deposit; consistent histologically with MM when resected. Postoperative PET-CT in August 2017 demonstrated local recurrence. In the setting of resected stage IV disease and a third local recurrence, the decision was made to instigate immunotherapy. Vulvar melanoma is rare accounting for 0.2% of all melanoma. Presentation is typically a decade later than cutaneous melanoma with a tendency to late metastases and poorer prognosis. Given their rarity the treatment paradigm is less clearly defined and largely extrapolated from that of cutaneous melanomas.</description><subject>Biopsy</subject><subject>Case reports</subject><subject>Colorectal cancer</subject><subject>Dermatology</subject><subject>Female</subject><subject>Gastrointestinal cancer</subject><subject>Gastrointestinal Stromal Tumors - diagnostic imaging</subject><subject>Gastrointestinal Stromal Tumors - drug therapy</subject><subject>Genetic disorders</subject><subject>Humans</subject><subject>Immunotherapy</subject><subject>Medical imaging</subject><subject>Melanoma</subject><subject>Melanoma - diagnostic imaging</subject><subject>Melanoma - drug therapy</subject><subject>Middle Aged</subject><subject>Mutation</subject><subject>Neoplasm Recurrence, Local - diagnostic imaging</subject><subject>Neoplasm Recurrence, Local - drug therapy</subject><subject>Neurofibromatoses - diagnostic imaging</subject><subject>Neurofibromatoses - drug therapy</subject><subject>Neurological disorders</subject><subject>Positron Emission Tomography Computed Tomography</subject><subject>Rare Disease</subject><subject>Skin cancer</subject><subject>Treatment Outcome</subject><subject>Tumors</subject><subject>Vulvar Neoplasms - diagnostic imaging</subject><subject>Vulvar Neoplasms - drug therapy</subject><issn>1757-790X</issn><issn>1757-790X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2019</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>BENPR</sourceid><recordid>eNqFkc1LBSEUxSWKilfrdiG0iWDKrzfqJojoC4IgCtqJ-pzyMTO-1HnRf5_Dq6g2uble7s_DPR4A9jA6xpjWJ8bGiiAsKkIYZ2wNbGM-5RWX6Gn9x30L7KY0R-VQzASjm2CLorqeSlJvA33v7BCj6zNcDu1SR9i5Vveh09D3UMOFzn4cvvn8Ans3xNB4E8s4h-QT1P0MPuuUY_B9din7XrdwbLtS89CFIe6AjUa3ye1-1gl4vLx4OL-ubu-ubs7PbivDMMqVlY0QZMapJNIYianhAhk-rQXRhZCIO8kMYoQI0RCrkdOWYEatlVY6JugEnK50F4Pp3MyWraNu1SL6Tsd3FbRXvye9f1HPYalqytC0rovA4adADK9DMaM6n6xry3e4MCRFMJcMc05YQQ_-oPPitHgfKck4Irw4mICTFWVjSCm65nsZjNSYoCoJqjFBtUqwvNj_6eGb_8qrAEcrwHTzf9U-AMlQpjI</recordid><startdate>20190101</startdate><enddate>20190101</enddate><creator>Linehan, Anna</creator><creator>Harrold, Emily</creator><creator>Pilson, Keith</creator><creator>McCaffrey, John</creator><general>BMJ Publishing Group LTD</general><general>BMJ Publishing Group</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>7RV</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>BTHHO</scope><scope>CCPQU</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9.</scope><scope>KB0</scope><scope>M0S</scope><scope>M1P</scope><scope>NAPCQ</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>20190101</creationdate><title>Recurrent vulvar melanoma in a patient with neurofibromatosis and gastrointestinal stromal tumour</title><author>Linehan, Anna ; Harrold, Emily ; Pilson, Keith ; McCaffrey, John</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-b410t-c9f882d73929bb913b780b75682ab41907e94b042288f2ca0eac2143cc9c9e483</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2019</creationdate><topic>Biopsy</topic><topic>Case reports</topic><topic>Colorectal cancer</topic><topic>Dermatology</topic><topic>Female</topic><topic>Gastrointestinal cancer</topic><topic>Gastrointestinal Stromal Tumors - diagnostic imaging</topic><topic>Gastrointestinal Stromal Tumors - drug therapy</topic><topic>Genetic disorders</topic><topic>Humans</topic><topic>Immunotherapy</topic><topic>Medical imaging</topic><topic>Melanoma</topic><topic>Melanoma - diagnostic imaging</topic><topic>Melanoma - drug therapy</topic><topic>Middle Aged</topic><topic>Mutation</topic><topic>Neoplasm Recurrence, Local - diagnostic imaging</topic><topic>Neoplasm Recurrence, Local - drug therapy</topic><topic>Neurofibromatoses - diagnostic imaging</topic><topic>Neurofibromatoses - drug therapy</topic><topic>Neurological disorders</topic><topic>Positron Emission Tomography Computed Tomography</topic><topic>Rare Disease</topic><topic>Skin cancer</topic><topic>Treatment Outcome</topic><topic>Tumors</topic><topic>Vulvar Neoplasms - diagnostic imaging</topic><topic>Vulvar Neoplasms - drug therapy</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Linehan, Anna</creatorcontrib><creatorcontrib>Harrold, Emily</creatorcontrib><creatorcontrib>Pilson, Keith</creatorcontrib><creatorcontrib>McCaffrey, John</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>Nursing & Allied Health Database</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</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</collection><collection>BMJ Journals</collection><collection>ProQuest One Community College</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Nursing & Allied Health Premium</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>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>BMJ case reports</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Linehan, Anna</au><au>Harrold, Emily</au><au>Pilson, Keith</au><au>McCaffrey, John</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Recurrent vulvar melanoma in a patient with neurofibromatosis and gastrointestinal stromal tumour</atitle><jtitle>BMJ case reports</jtitle><addtitle>BMJ Case Rep</addtitle><date>2019-01-01</date><risdate>2019</risdate><volume>12</volume><issue>1</issue><spage>e224744</spage><pages>e224744-</pages><issn>1757-790X</issn><eissn>1757-790X</eissn><abstract>We report a case of a 51-year-old woman with neurofibromatosis who presented in 2012 with postmenopausal bleeding. Excision biopsy of a pigmented lesion of the labia minora was consistent with an ulcerated vulvar BRAF wild type malignant melanoma (MM). Initial excision was followed by radical vulvectomy and adjuvant interferon. Local recurrence in January 2017 was further resected. Positron emission tomography (PET)-CT in May 2017 identified an FDG avid omental deposit; consistent histologically with MM when resected. Postoperative PET-CT in August 2017 demonstrated local recurrence. In the setting of resected stage IV disease and a third local recurrence, the decision was made to instigate immunotherapy. Vulvar melanoma is rare accounting for 0.2% of all melanoma. Presentation is typically a decade later than cutaneous melanoma with a tendency to late metastases and poorer prognosis. Given their rarity the treatment paradigm is less clearly defined and largely extrapolated from that of cutaneous melanomas.</abstract><cop>England</cop><pub>BMJ Publishing Group LTD</pub><pmid>30665926</pmid><doi>10.1136/bcr-2018-224744</doi><oa>free_for_read</oa></addata></record> |
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subjects | Biopsy Case reports Colorectal cancer Dermatology Female Gastrointestinal cancer Gastrointestinal Stromal Tumors - diagnostic imaging Gastrointestinal Stromal Tumors - drug therapy Genetic disorders Humans Immunotherapy Medical imaging Melanoma Melanoma - diagnostic imaging Melanoma - drug therapy Middle Aged Mutation Neoplasm Recurrence, Local - diagnostic imaging Neoplasm Recurrence, Local - drug therapy Neurofibromatoses - diagnostic imaging Neurofibromatoses - drug therapy Neurological disorders Positron Emission Tomography Computed Tomography Rare Disease Skin cancer Treatment Outcome Tumors Vulvar Neoplasms - diagnostic imaging Vulvar Neoplasms - drug therapy |
title | Recurrent vulvar melanoma in a patient with neurofibromatosis and gastrointestinal stromal tumour |
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