{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,3]],"date-time":"2026-09-03T12:41:48Z","timestamp":1788439308488,"version":"build-2803163510"},"reference-count":13,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2018,1,3]],"date-time":"2018-01-03T00:00:00Z","timestamp":1514937600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2018,6]]},"abstract":"<jats:p>We report a case of low\u2010grade fibromyxoid sarcoma arising within the median nerve. A 31\u2010year\u2010old woman presented with symptoms of carpal tunnel syndrome and an enlarging mass in her right palm over 1\u2009year. MRI demonstrated a mass associated with the right median nerve with solid and cystic components. At surgery, the mass was located within the epineurium, could be bluntly dissected from the nerve fascicles, and was suspected to be a schwannoma. A 3.4\u2009cm, tan\u2010pink, glistening, smooth, homogenous mass was submitted to pathology. Microscopically, the tumor was a solid and cystic circumscribed nodule with a dense fibrous pseudocapsule. The tumor cells were uniformly bland and spindle\u2010shaped, with small, hyperchromatic oval nuclei and were embedded in an alternating fibrous and myxoid stroma with a prominent curvilinear vasculature and perivascular sclerosis. The differential diagnosis for this lesion included myxoid neurofibroma, schwannoma, soft tissue perineurioma, low\u2010grade malignant peripheral nerve sheath tumor and low\u2010grade fibromyxoid sarcoma. The tumor cells expressed MUC4, GLUT\u20101, and vimentin and were negative for S\u2010100 protein, epithelial membrane antigen, smooth muscle actin, desmin, claudin\u20101, neurofilament and SOX10. Fluorescence in situ hybridization, with a break\u2010apart probe strategy, demonstrated <jats:italic>FUS<\/jats:italic> rearrangement, consistent in this morphological context with the low\u2010grade fibromyxoid sarcoma\u2010associated <jats:italic>FUS\u2010CREB3L2<\/jats:italic> or <jats:italic>FUS\u2010CREB3L1<\/jats:italic> fusions. Low\u2010grade fibromyxoid sarcoma is exceptionally rare in the peripheral nerve, with only a single previously reported case. Nonetheless, as our case illustrates, this entity must be included in the differential diagnosis of unusual intraneural mesenchymal tumors. As in all other locations, intraneural low\u2010grade fibromyxoid sarcomas should be excised with negative margins. Patients with this disease require long\u2010term clinical follow\u2010up, given this tumor's propensity for very late distant metastases to the lungs and other sites.<\/jats:p>","DOI":"10.1111\/neup.12453","type":"journal-article","created":{"date-parts":[[2018,1,3]],"date-time":"2018-01-03T12:37:14Z","timestamp":1514983034000},"page":"309-314","source":"Crossref","is-referenced-by-count":4,"title":["Low\u2010grade fibromyxoid sarcoma arising within the median nerve"],"prefix":"10.1111","volume":"38","author":[{"given":"Amy A.","family":"Swanson","sequence":"first","affiliation":[{"name":"Department of Laboratory Medicine and Anatomic Pathology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2757-6782","authenticated-orcid":false,"given":"Caterina","family":"Giannini","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Anatomic Pathology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew L.","family":"Folpe","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Anatomic Pathology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel L.","family":"Van Dyke","sequence":"additional","affiliation":[{"name":"Department of Laboratory Genetics and Genomics Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kimberly K.","family":"Amrami","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Radiology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William A.","family":"Michalak","sequence":"additional","affiliation":[{"name":"Department of Pathology Butler Memorial Hospital  Butler Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachael A.","family":"Vaubel","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Anatomic Pathology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,1,3]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2010.08.019"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-012-0954-z"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.3171\/2014.3.JNS13838"},{"key":"e_1_2_7_5_1","doi-asserted-by":"crossref","first-page":"553","DOI":"10.55418\/9781933477305","volume-title":"AFIP Atlas of Tumor Pathology\u2014Tumors of the Peripheral Nervous System (Fascicle 19)","author":"Antonescu CR","year":"2013"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcp\/88.5.615"},{"key":"e_1_2_7_7_1","first-page":"324","volume-title":"Enzinger and Weiss's Soft Tissue Tumors","author":"Goldblum JR","year":"2014"},{"key":"e_1_2_7_8_1","first-page":"95","volume-title":"WHO Classification of Tumours of Soft Tissue and Bone","author":"Folpe AL","year":"2013"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-200010000-00004"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-199712000-00011"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e318210c268"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/his.12282"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3181578d5a"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e31822b3687"}],"container-title":["Neuropathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fneup.12453","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/neup.12453","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,23]],"date-time":"2023-09-23T12:51:20Z","timestamp":1695473480000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/neup.12453"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,1,3]]},"references-count":13,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2018,6]]}},"alternative-id":["10.1111\/neup.12453"],"URL":"https:\/\/doi.org\/10.1111\/neup.12453","archive":["Portico"],"relation":{},"ISSN":["0919-6544","1440-1789"],"issn-type":[{"value":"0919-6544","type":"print"},{"value":"1440-1789","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,1,3]]}}}