Clinical, pathologic, and molecular analyses of superficial low‐grade fibromyxoid sarcoma in two young patients: A rare and deceptive mimic of benignancy
Low‐grade fibromyxoid sarcoma (LGFMS) is a histopathologically deceptive soft tissue neoplasm with bland cytology, which is typically encountered in deep soft tissue of adults. We report two cases of superficial LGFMS in young patients (16 and 21 years old, respectively), which were difficult to dia...
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Veröffentlicht in: | Journal of cutaneous pathology 2022-07, Vol.49 (7), p.638-644 |
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description | Low‐grade fibromyxoid sarcoma (LGFMS) is a histopathologically deceptive soft tissue neoplasm with bland cytology, which is typically encountered in deep soft tissue of adults. We report two cases of superficial LGFMS in young patients (16 and 21 years old, respectively), which were difficult to diagnose on histopathologic and clinical findings alone. LGFMS commonly mimics benign neoplasms such as cellular neurothekeoma, fibromatosis, neurofibroma, and perineurioma. Malignancies included in the differential diagnosis are soft tissue neoplasms such as dermatofibrosarcoma protuberans and myxofibrosarcoma. A high degree of reported variation in pattern and cellularity among LGFMS further complicates the diagnosis. Careful examination and appropriate immunohistochemistry panels including MUC4 are essential for narrowing the differential diagnosis. Molecular studies for possible FUS translocation can confirm the diagnosis of LGFMS. Sufficient sampling and workup of these lesions are critical, especially in younger patients. Young age and superficial presentation can easily sway dermatopathologists/dermatologists toward an incorrect diagnosis of benignancy. |
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We report two cases of superficial LGFMS in young patients (16 and 21 years old, respectively), which were difficult to diagnose on histopathologic and clinical findings alone. LGFMS commonly mimics benign neoplasms such as cellular neurothekeoma, fibromatosis, neurofibroma, and perineurioma. Malignancies included in the differential diagnosis are soft tissue neoplasms such as dermatofibrosarcoma protuberans and myxofibrosarcoma. A high degree of reported variation in pattern and cellularity among LGFMS further complicates the diagnosis. Careful examination and appropriate immunohistochemistry panels including MUC4 are essential for narrowing the differential diagnosis. Molecular studies for possible FUS translocation can confirm the diagnosis of LGFMS. Sufficient sampling and workup of these lesions are critical, especially in younger patients. Young age and superficial presentation can easily sway dermatopathologists/dermatologists toward an incorrect diagnosis of benignancy.</description><identifier>ISSN: 0303-6987</identifier><identifier>EISSN: 1600-0560</identifier><identifier>DOI: 10.1111/cup.14215</identifier><identifier>PMID: 35191077</identifier><language>eng</language><publisher>Oxford, UK: Blackwell Publishing Ltd</publisher><subject>Adolescent ; Adult ; Cytology ; Dermatofibrosarcoma protuberans ; Differential diagnosis ; Fibroma - diagnosis ; Fibroma - pathology ; Fibrosarcoma - diagnosis ; Fibrosarcoma - pathology ; FUS translocation ; Humans ; Immunohistochemistry ; MUC4 ; Neoplasia ; Nerve Sheath Neoplasms ; Patients ; pediatric ; Sarcoma ; soft tissue neoplasm ; Soft Tissue Neoplasms - pathology ; superficial low‐grade fibromyxoid sarcoma ; Young Adult</subject><ispartof>Journal of cutaneous pathology, 2022-07, Vol.49 (7), p.638-644</ispartof><rights>2022 John Wiley & Sons A/S. 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Published by John Wiley & Sons Ltd.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4105-a87035ebb151bbab07b15186597f0fda7fb617281461a1ae19cfe996ea8b718d3</citedby><cites>FETCH-LOGICAL-c4105-a87035ebb151bbab07b15186597f0fda7fb617281461a1ae19cfe996ea8b718d3</cites><orcidid>0000-0002-8913-4886 ; 0000-0003-0383-031X ; 0000-0001-9267-9484</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fcup.14215$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fcup.14215$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>230,314,778,782,883,1414,27907,27908,45557,45558</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/35191077$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Modi, Mitul B.</creatorcontrib><creatorcontrib>Pei, Susan</creatorcontrib><creatorcontrib>Hedberg, Matthew L.</creatorcontrib><creatorcontrib>Fischer, Andrew S.</creatorcontrib><creatorcontrib>Guerrero, Jessenia</creatorcontrib><creatorcontrib>Carreon, Chrystalle Katte</creatorcontrib><creatorcontrib>Cooper, Kumarasen</creatorcontrib><creatorcontrib>Seykora, John T.</creatorcontrib><title>Clinical, pathologic, and molecular analyses of superficial low‐grade fibromyxoid sarcoma in two young patients: A rare and deceptive mimic of benignancy</title><title>Journal of cutaneous pathology</title><addtitle>J Cutan Pathol</addtitle><description>Low‐grade fibromyxoid sarcoma (LGFMS) is a histopathologically deceptive soft tissue neoplasm with bland cytology, which is typically encountered in deep soft tissue of adults. We report two cases of superficial LGFMS in young patients (16 and 21 years old, respectively), which were difficult to diagnose on histopathologic and clinical findings alone. LGFMS commonly mimics benign neoplasms such as cellular neurothekeoma, fibromatosis, neurofibroma, and perineurioma. Malignancies included in the differential diagnosis are soft tissue neoplasms such as dermatofibrosarcoma protuberans and myxofibrosarcoma. A high degree of reported variation in pattern and cellularity among LGFMS further complicates the diagnosis. Careful examination and appropriate immunohistochemistry panels including MUC4 are essential for narrowing the differential diagnosis. Molecular studies for possible FUS translocation can confirm the diagnosis of LGFMS. Sufficient sampling and workup of these lesions are critical, especially in younger patients. Young age and superficial presentation can easily sway dermatopathologists/dermatologists toward an incorrect diagnosis of benignancy.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Cytology</subject><subject>Dermatofibrosarcoma protuberans</subject><subject>Differential diagnosis</subject><subject>Fibroma - diagnosis</subject><subject>Fibroma - pathology</subject><subject>Fibrosarcoma - diagnosis</subject><subject>Fibrosarcoma - pathology</subject><subject>FUS translocation</subject><subject>Humans</subject><subject>Immunohistochemistry</subject><subject>MUC4</subject><subject>Neoplasia</subject><subject>Nerve Sheath Neoplasms</subject><subject>Patients</subject><subject>pediatric</subject><subject>Sarcoma</subject><subject>soft tissue neoplasm</subject><subject>Soft Tissue Neoplasms - pathology</subject><subject>superficial low‐grade fibromyxoid sarcoma</subject><subject>Young Adult</subject><issn>0303-6987</issn><issn>1600-0560</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2022</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp1kcFu1DAQhi0EokvhwAsgS5yQmtZONnbCAalaQUGqVA70bI2dSerKiYOddMmNR-DO2_Ek9XZLRQ_4Yo_86Zuxf0Jec3bM0zox83jM1zkvn5AVF4xlrBTsKVmxghWZqCt5QF7EeM0YF5Uon5ODouQ1Z1KuyO-Ns4M14I7oCNOVd76z5ojC0NDeOzSzg5AqcEvESH1L4zxiaK2x4Kjz2z8_f3UBGqSt1cH3yw9vGxohGN8DtQOdtp4ufh66nd7iMMX39JQGCHjXo0GD42RvkPa2t2bXQONguwEGs7wkz1pwEV_d74fk8tPHb5vP2fnF2ZfN6Xlm1pyVGVSSFSVqzUuuNWgmd6f00Fq2rG1AtlpwmVd8LThwQF6bFutaIFRa8qopDsmHvXecdY-NSVMGcGoMtoewKA9WPb4Z7JXq_I2q8yKvqyIJ3t4Lgv8-Y5zUtZ9D-rSociFlLtdMVol6t6dM8DEGbB86cKZ2OaqUo7rLMbFv_h3pgfwbXAJO9sDWOlz-b1Kby6975S1pZKxL</recordid><startdate>202207</startdate><enddate>202207</enddate><creator>Modi, Mitul B.</creator><creator>Pei, Susan</creator><creator>Hedberg, Matthew L.</creator><creator>Fischer, Andrew S.</creator><creator>Guerrero, Jessenia</creator><creator>Carreon, Chrystalle Katte</creator><creator>Cooper, Kumarasen</creator><creator>Seykora, John T.</creator><general>Blackwell Publishing Ltd</general><general>Wiley Subscription Services, Inc</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>7T5</scope><scope>7TM</scope><scope>7TO</scope><scope>7U9</scope><scope>H94</scope><scope>5PM</scope><orcidid>https://orcid.org/0000-0002-8913-4886</orcidid><orcidid>https://orcid.org/0000-0003-0383-031X</orcidid><orcidid>https://orcid.org/0000-0001-9267-9484</orcidid></search><sort><creationdate>202207</creationdate><title>Clinical, pathologic, and molecular analyses of superficial low‐grade fibromyxoid sarcoma in two young patients: A rare and deceptive mimic of benignancy</title><author>Modi, Mitul B. ; Pei, Susan ; Hedberg, Matthew L. ; Fischer, Andrew S. ; Guerrero, Jessenia ; Carreon, Chrystalle Katte ; Cooper, Kumarasen ; Seykora, John T.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4105-a87035ebb151bbab07b15186597f0fda7fb617281461a1ae19cfe996ea8b718d3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2022</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Cytology</topic><topic>Dermatofibrosarcoma protuberans</topic><topic>Differential diagnosis</topic><topic>Fibroma - diagnosis</topic><topic>Fibroma - pathology</topic><topic>Fibrosarcoma - diagnosis</topic><topic>Fibrosarcoma - pathology</topic><topic>FUS translocation</topic><topic>Humans</topic><topic>Immunohistochemistry</topic><topic>MUC4</topic><topic>Neoplasia</topic><topic>Nerve Sheath Neoplasms</topic><topic>Patients</topic><topic>pediatric</topic><topic>Sarcoma</topic><topic>soft tissue neoplasm</topic><topic>Soft Tissue Neoplasms - pathology</topic><topic>superficial low‐grade fibromyxoid sarcoma</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Modi, Mitul B.</creatorcontrib><creatorcontrib>Pei, Susan</creatorcontrib><creatorcontrib>Hedberg, Matthew L.</creatorcontrib><creatorcontrib>Fischer, Andrew S.</creatorcontrib><creatorcontrib>Guerrero, Jessenia</creatorcontrib><creatorcontrib>Carreon, Chrystalle Katte</creatorcontrib><creatorcontrib>Cooper, Kumarasen</creatorcontrib><creatorcontrib>Seykora, John T.</creatorcontrib><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>Nucleic Acids Abstracts</collection><collection>Oncogenes and Growth Factors Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Journal of cutaneous pathology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Modi, Mitul B.</au><au>Pei, Susan</au><au>Hedberg, Matthew L.</au><au>Fischer, Andrew S.</au><au>Guerrero, Jessenia</au><au>Carreon, Chrystalle Katte</au><au>Cooper, Kumarasen</au><au>Seykora, John T.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Clinical, pathologic, and molecular analyses of superficial low‐grade fibromyxoid sarcoma in two young patients: A rare and deceptive mimic of benignancy</atitle><jtitle>Journal of cutaneous pathology</jtitle><addtitle>J Cutan Pathol</addtitle><date>2022-07</date><risdate>2022</risdate><volume>49</volume><issue>7</issue><spage>638</spage><epage>644</epage><pages>638-644</pages><issn>0303-6987</issn><eissn>1600-0560</eissn><abstract>Low‐grade fibromyxoid sarcoma (LGFMS) is a histopathologically deceptive soft tissue neoplasm with bland cytology, which is typically encountered in deep soft tissue of adults. We report two cases of superficial LGFMS in young patients (16 and 21 years old, respectively), which were difficult to diagnose on histopathologic and clinical findings alone. LGFMS commonly mimics benign neoplasms such as cellular neurothekeoma, fibromatosis, neurofibroma, and perineurioma. Malignancies included in the differential diagnosis are soft tissue neoplasms such as dermatofibrosarcoma protuberans and myxofibrosarcoma. A high degree of reported variation in pattern and cellularity among LGFMS further complicates the diagnosis. Careful examination and appropriate immunohistochemistry panels including MUC4 are essential for narrowing the differential diagnosis. Molecular studies for possible FUS translocation can confirm the diagnosis of LGFMS. Sufficient sampling and workup of these lesions are critical, especially in younger patients. Young age and superficial presentation can easily sway dermatopathologists/dermatologists toward an incorrect diagnosis of benignancy.</abstract><cop>Oxford, UK</cop><pub>Blackwell Publishing Ltd</pub><pmid>35191077</pmid><doi>10.1111/cup.14215</doi><tpages>7</tpages><orcidid>https://orcid.org/0000-0002-8913-4886</orcidid><orcidid>https://orcid.org/0000-0003-0383-031X</orcidid><orcidid>https://orcid.org/0000-0001-9267-9484</orcidid><oa>free_for_read</oa></addata></record> |
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subjects | Adolescent Adult Cytology Dermatofibrosarcoma protuberans Differential diagnosis Fibroma - diagnosis Fibroma - pathology Fibrosarcoma - diagnosis Fibrosarcoma - pathology FUS translocation Humans Immunohistochemistry MUC4 Neoplasia Nerve Sheath Neoplasms Patients pediatric Sarcoma soft tissue neoplasm Soft Tissue Neoplasms - pathology superficial low‐grade fibromyxoid sarcoma Young Adult |
title | Clinical, pathologic, and molecular analyses of superficial low‐grade fibromyxoid sarcoma in two young patients: A rare and deceptive mimic of benignancy |
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