{"title":"A locally advanced PML::JAK1 myofibroblastic sarcoma arising in the neck.","authors":"Saeko Soejima, Jotaro Nakashima, Naoki Kojima, Miyako Kitazono, Dai Inoue, Shunji Takahashi, Haruka Okada","doi":"10.1007/s00428-026-04744-3","DOIUrl":null,"url":null,"abstract":"<p><p>A 54-year-old male patient presented with a painful right cervical mass encasing the common carotid artery. Histologically, the tumor consisted of polygonal to spindle-shaped cells with mild nuclear atypia and low mitotic activity (0-1/10 high-power fields). No myxoid stroma or necrosis was identified, and desmin expression was patchy. GenMineTOP, a dual DNA-RNA comprehensive genomic profiling system, revealed a PML exon 7-JAK1 exon 5 fusion. The tumor was surgically unresectable because of circumferential encasement of the right common carotid artery. After radiotherapy followed by doxorubicin chemotherapy, no distant metastases developed during 17 months of follow-up. PML::JAK1 myofibroblastic sarcomas have previously been reported in four abdominal cases, most of which showed aggressive behavior. This cervical case broadens the anatomic spectrum of PML::JAK1 myofibroblastic sarcomas to include the head and neck region and further expands their morphologic spectrum.</p>","PeriodicalId":23514,"journal":{"name":"Virchows Archiv","volume":" ","pages":""},"PeriodicalIF":3.0000,"publicationDate":"2026-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Virchows Archiv","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s00428-026-04744-3","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"PATHOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
A 54-year-old male patient presented with a painful right cervical mass encasing the common carotid artery. Histologically, the tumor consisted of polygonal to spindle-shaped cells with mild nuclear atypia and low mitotic activity (0-1/10 high-power fields). No myxoid stroma or necrosis was identified, and desmin expression was patchy. GenMineTOP, a dual DNA-RNA comprehensive genomic profiling system, revealed a PML exon 7-JAK1 exon 5 fusion. The tumor was surgically unresectable because of circumferential encasement of the right common carotid artery. After radiotherapy followed by doxorubicin chemotherapy, no distant metastases developed during 17 months of follow-up. PML::JAK1 myofibroblastic sarcomas have previously been reported in four abdominal cases, most of which showed aggressive behavior. This cervical case broadens the anatomic spectrum of PML::JAK1 myofibroblastic sarcomas to include the head and neck region and further expands their morphologic spectrum.
期刊介绍：
Manuscripts of original studies reinforcing the evidence base of modern diagnostic pathology, using immunocytochemical, molecular and ultrastructural techniques, will be welcomed. In addition, papers on critical evaluation of diagnostic criteria but also broadsheets and guidelines with a solid evidence base will be considered. Consideration will also be given to reports of work in other fields relevant to the understanding of human pathology as well as manuscripts on the application of new methods and techniques in pathology. Submission of purely experimental articles is discouraged but manuscripts on experimental work applicable to diagnostic pathology are welcomed. Biomarker studies are welcomed but need to abide by strict rules (e.g. REMARK) of adequate sample size and relevant marker choice. Single marker studies on limited patient series without validated application will as a rule not be considered. Case reports will only be considered when they provide substantial new information with an impact on understanding disease or diagnostic practice.
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