{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,17]],"date-time":"2026-06-17T02:35:37Z","timestamp":1781663737945,"version":"3.54.5"},"reference-count":27,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2024,9,9]],"date-time":"2024-09-09T00:00:00Z","timestamp":1725840000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,2,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Objectives<\/jats:title>\n                  <jats:p>Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue neoplasm of uncertain histogenesis. Most OFMTs have benign behavior, and many harbor gene fusions involving the PHD finger protein 1 (PHF1), such as EP400::PHF1, MEAF6::PHF1, EPC1::PHF1, and PHF1::TFE3. The PHF1::TFE3 fusion is unique because PHF1 is at 5\u02b9 instead of residing at 3\u02b9 in the other fusions. In this study, we describe 2 cases of OFMT harboring PHF1::TFE3 fusions and review 13 published cases.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>Two cases of PHF1::TFE3-positive OFMT were investigated using RNA Next-Generation Sequencing and immunohistochemistry.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Most (12\/15) of the PHF1::TFE3 OFMTs we studied were located at proximal and distal extremities, with a multinodular growth pattern. Only 1 case (1\/10) had a shell of bone at the periphery. Areas morphologically similar to sclerosing epithelioid fibrosarcoma or low-grade fibromyxoid sarcoma were found in 8 of 12 (66.7%) cases. Eleven cases (11\/15 [73.3%]) were regarded as malignant based on more than 2\/50 high-power field mitotic figures, increased cellularity, or the presence of necrosis. Among the 9 cases with follow-up data, 2 patients died of disease (with metastases), 1 patient is alive with metastases, and 1 patient had multiple local recurrences.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>Because PHF1 is located at 3\u02b9 in all the PHF1 fusions in OFMTs except PHF1::TFE3, the different driver molecular alterations suggest that OFMTs with 3\u02b9-PHF1 fusions and OFMTs with PHF1::TFE3 are different tumors. Immunohistochemistry confirmed TFE3 expression in all PHF1::TFE3 OFMTs. Because PHF1::TFE3-positive OFMTs have increased mitotic figures and tumor cellularity, with a high rate of metastasis, using the name PHF1::TFE3 positive fibromyxoid sarcoma may be appropriate.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ajcp\/aqae114","type":"journal-article","created":{"date-parts":[[2024,9,9]],"date-time":"2024-09-09T20:09:10Z","timestamp":1725912550000},"page":"224-230","source":"Crossref","is-referenced-by-count":5,"title":["<i>PHF1::TFE3-<\/i>positive fibromyxoid sarcoma? Report of 2 cases and review of 13 cases of <i>PHF1::TFE3-<\/i>positive ossifying fibromyxoid tumor in the literature"],"prefix":"10.1093","volume":"163","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0441-6973","authenticated-orcid":false,"given":"Shuanzeng","family":"Wei","sequence":"first","affiliation":[{"name":"Department of Pathology, Fox Chase Cancer Center , Philadelphia, PA ,","place":["US"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arthur S","family":"Patchefsky","sequence":"additional","affiliation":[{"name":"Department of Pathology, Fox Chase Cancer Center , Philadelphia, PA ,","place":["US"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jianming","family":"Pei","sequence":"additional","affiliation":[{"name":"Department of Pathology, Fox Chase Cancer Center , Philadelphia, PA ,","place":["US"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scot A","family":"Brown","sequence":"additional","affiliation":[{"name":"Department of Orthopedic Oncology, Rothman orthopedics , Philadelphia, PA ,","place":["US"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atrayee","family":"Basu Mallick","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, Thomas Jefferson University Hospital , Philadelphia, PA ,","place":["US"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zixuan","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Pathology and Genomic Medicine, Thomas Jefferson University Hospital , Philadelphia, PA ,","place":["US"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei","family":"Jiang","sequence":"additional","affiliation":[{"name":"Department of Pathology and Genomic Medicine, Thomas Jefferson University Hospital , Philadelphia, PA ,","place":["US"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2024,9,9]]},"reference":[{"issue":"6","key":"2025021323430349000_CIT0001","doi-asserted-by":"publisher","first-page":"709","DOI":"10.1097\/PAS.0000000000002036","article-title":"Ossifying fibromyxoid tumor of the genitourinary tract: report of 4 molecularly confirmed cases of a diagnostic pitfall","volume":"47","author":"Argani","year":"2023","journal-title":"Am J Surg Pathol."},{"issue":"4","key":"2025021323430349000_CIT0002","doi-asserted-by":"publisher","first-page":"421","DOI":"10.1097\/00000478-200304000-00001","article-title":"Ossifying fibromyxoid tumor of soft parts: a clinicopathologic study of 70 cases with emphasis on atypical and malignant variants","volume":"27","author":"Folpe","year":"2003","journal-title":"Am J Surg Pathol."},{"issue":"10","key":"2025021323430349000_CIT0003","doi-asserted-by":"publisher","first-page":"817","DOI":"10.1097\/00000478-198910000-00001","article-title":"Ossifying fibromyxoid tumor of soft parts. 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