{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T08:28:04Z","timestamp":1787646484718,"version":"build-2736575974"},"reference-count":23,"publisher":"SAGE Publications","issue":"4","license":[{"start":{"date-parts":[[2023,8,7]],"date-time":"2023-08-07T00:00:00Z","timestamp":1691366400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"funder":[{"name":"Basic Research and Frontier exploration project of Chongqing Yuzhong District","award":["20210163"],"award-info":[{"award-number":["20210163"]}]}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Int J Surg Pathol"],"published-print":{"date-parts":[[2024,6]]},"abstract":"<jats:p>\n                    Rhabdomyosarcoma (RMS) with TFCP2 rearrangement has been identified recently. This entity has a distinctive clinicopathologic features: a rapidly aggressive clinical course, a preference for the craniofacial bones, a spindle and epithelioid histomorphology, and positive immunohistochemistry for epithelial markers, ALK, and myogenic markers. RMS with TFCP2 rearrangement is rare and may be misdiagnosed as other spindle cell tumors. Here, we report a case of this entity arising in the mandible, which was initially diagnosed as ossifying fibroma in primary tumor in another hospital. A 26-year-old man presented with a recurred mass in the mandible for 1 month after the operation of mandibular tumor. The first excisional specimen was initially diagnosed as ossifying fibroma in another hospital. Histopathologic examination revealed the tumor with a hybrid spindle cell and epithelioid cytomorphology, spindle cells and spindle-to-epithelioid cells with eosinophilic and rich cytoplasm, with high-grade features, prominent nucleoli and some atypical mitosis. Immunohistochemical analysis revealed positivity for desmin, MYOD1, pan-keratin, ALK (5A4), ALK (D5F3). Based on the morphology and immunophenotype, molecular studies were performed, which revealed a\n                    <jats:italic toggle=\"yes\">FUS::TFCP2<\/jats:italic>\n                    fusion transcript, confirming the diagnosis of Rhabdomyosarcoma with\n                    <jats:italic toggle=\"yes\">FUS::TFCP2<\/jats:italic>\n                    fusion. Making a correct diagnosis is primarily dependent on awareness by the pathologist of this rare subtype of RMS and careful histopathological evaluation, supported by immunohistochemical and molecular analysis, to avoid potential diagnostic pitfalls.\n                  <\/jats:p>","DOI":"10.1177\/10668969231189172","type":"journal-article","created":{"date-parts":[[2023,8,7]],"date-time":"2023-08-07T03:10:41Z","timestamp":1691377841000},"page":"758-766","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":5,"title":["Rhabdomyosarcoma With\n                    <i>FUS::TFCP2<\/i>\n                    Fusion in the Mandible: A Rare Aggressive Subtype, but Can Be Misdiagnosed as Ossifying Fibroma"],"prefix":"10.1177","volume":"32","author":[{"given":"Peng","family":"Zhong","sequence":"first","affiliation":[{"name":"Department of Pathology, Daping Hospital, Amy Medical University (Third Military Medical University), Chongqing, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shirong","family":"Wei","sequence":"additional","affiliation":[{"name":"Department of Pathology, Daping Hospital, Amy Medical University (Third Military Medical University), Chongqing, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hualiang","family":"Xiao","sequence":"additional","affiliation":[{"name":"Department of Pathology, Daping Hospital, Amy Medical University (Third Military Medical University), Chongqing, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7428-4382","authenticated-orcid":false,"given":"Ying","family":"Zeng","sequence":"additional","affiliation":[{"name":"Department of Pathology, Chongqing General Hospital, Chongqing, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2023,8,7]]},"reference":[{"key":"e_1_3_2_2_2","volume-title":"World Health Organization classification of soft tissue and bone tumours","author":"WHO Classification of Tumours Editorial Board","year":"2020","unstructured":"WHO Classification of Tumours Editorial Board. World Health Organization classification of soft tissue and bone tumours. 5th ed. IARC Press; 2020.","edition":"5"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1002\/path.5053"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1111\/his.13649"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000001227"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.prp.2019.03.027"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.pathol.2018.09.056"},{"key":"e_1_3_2_8_2","doi-asserted-by":"crossref","unstructured":"Brunac AC Laprie A Castex MP et al. The combination of radiotherapy and ALK inhibitors is effective in the treatment of intraosseous rhabdomyosarcoma with FUS-TFCP2 fusion transcript. Pediatr Blood Cancer. 2020;67(5):e28185.","DOI":"10.1002\/pbc.28185"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00428-020-02870-0"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41379-019-0323-8"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1007\/s12105-020-01189-1"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1111\/his.14323"},{"issue":"6","key":"e_1_3_2_13_2","first-page":"545","article-title":"[Clinicopathological features of rhabdomyosarcoma with TFCP2 fusions]","volume":"51","year":"2022","unstructured":"Chen XY, Chen G, Zhu Q, Zhu WF, He C, Huang RF. [Clinicopathological features of rhabdomyosarcoma with TFCP2 fusions]. Zhonghua Bing Li Xue Za Zhi. 2022;51(6):545-547.","journal-title":"Zhonghua Bing Li Xue Za Zhi"},{"key":"e_1_3_2_14_2","doi-asserted-by":"crossref","unstructured":"Duan FL Yang H Gong X et al. Clinicopathological features of rhabdomyosarcoma with novel FET::TFCP2 and TIMP3::ALK fusion: report of two cases and literature review. Histopathology. 2023;82(3):478-484.","DOI":"10.1111\/his.14833"},{"key":"e_1_3_2_15_2","volume-title":"Head Neck Pathol.","unstructured":"Gallagher KPD, Roza ALOC, Tager EMJR, et al. Rhabdomyosarcoma with TFCP2 rearrangement or typical co-expression of AE1\/AE3 and ALK: report of three new cases in the head and neck region and literature review. Head Neck Pathol. 2023;17(2):546-561."},{"key":"e_1_3_2_16_2","doi-asserted-by":"crossref","unstructured":"Ishiyama T Kato I Ito J et al. Rhabdomyosarcoma with FUS::TFCP2 fusion in the scalp: a rare case report depicting round and spindle cell morphology. Int J Surg Pathol. 2023;31(5):805-812.","DOI":"10.1177\/10668969221137517"},{"key":"e_1_3_2_17_2","doi-asserted-by":"publisher","DOI":"10.1007\/s12105-022-01424-x"},{"key":"e_1_3_2_18_2","doi-asserted-by":"crossref","unstructured":"Panferova A Sinichenkova KY Abu Jabal M et al. EWSR1-TFCP2 in an adolescent represents an extremely rare and aggressive form of intraosseous spindle cell rhabdomyosarcomas. Cold Spring Harb Mol Case Stud. 2022;8(5):a006209.","DOI":"10.1101\/mcs.a006209"},{"key":"e_1_3_2_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.oraloncology.2022.105876"},{"key":"e_1_3_2_20_2","doi-asserted-by":"crossref","unstructured":"Val\u00e9rio E Furtado Costa JL Perez Fraile NM et al. Intraosseous spindle cell\/epithelioid rhabdomyosarcoma with TFCP2 rearrangement: a recent recognized subtype with partial response to alectinib. 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