{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,28]],"date-time":"2026-07-28T18:14:10Z","timestamp":1785262450066,"version":"3.55.0"},"reference-count":49,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2025,4,29]],"date-time":"2025-04-29T00:00:00Z","timestamp":1745884800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Background: Ossifying fibromyxoid tumors (OFMTs) are rare mesenchymal neoplasms with behaviors ranging from benign to malignant. Although most occur in the extremities and trunk, 9\u201313% are found in the head and neck, such as the oral cavity, scalp, and calvarium. Diagnosis is challenging due to their rarity and histological similarity to other neoplasms. This review synthesizes evidence on the clinical presentation, diagnostic features, and treatment outcomes of OFMTs in the head and neck, focusing on surgical management and adjuvant therapies. Methods: A systematic review was conducted according to PRISMA guidelines, with searches in PubMed\/MEDLINE, Embase, Scopus, and Web of Science. Studies from 1989 to 2024 reporting OFMTs in the head and neck with clinical, histopathological, and treatment data were included. Extracted data encompassed demographics, tumor features, surgical margins, adjuvant therapy, and outcomes. Results: Forty studies with 99 patients were included. Patient ages ranged from 3 weeks to 88 years (median 47), with a male predominance (63.64%). The most common presentation was a slow-growing, painless mass. Tumors were most often found in the neck, oral cavity, scalp, and calvarium. Histopathology revealed encapsulated tumors with fibromyxoid stroma, spindle-shaped cells, and a peripheral rim of metaplastic bone in 70% of cases. Immunohistochemistry showed positivity for S-100, vimentin, and SOX10. Surgical excision was the main treatment, used in 28.28% of cases, with recurrence in 9.09% and no metastases. Adjuvant therapies, mainly radiotherapy, were employed in 15.15% of cases. Conclusions: OFMTs of the head and neck are rare neoplasms requiring multidisciplinary care. Imaging, histopathology, and immunohistochemistry are key to diagnosis. Surgical excision with clear margins remains the primary treatment, with a low recurrence rate. Adjuvant therapy may be needed for malignant or incompletely excised cases. Further research is needed to optimize follow-up protocols and assess molecular profiling for risk stratification.<\/jats:p>","DOI":"10.3390\/cancers17091508","type":"journal-article","created":{"date-parts":[[2025,4,30]],"date-time":"2025-04-30T05:05:57Z","timestamp":1745989557000},"page":"1508","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Ossifying Fibromyxoid Tumor of Soft Parts in the Head and Neck: A Systematic Review Addressing Surgical Management and Adjuvant Therapies"],"prefix":"10.3390","volume":"17","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9465-2506","authenticated-orcid":false,"given":"Gianluca","family":"Scalia","sequence":"first","affiliation":[{"name":"Neurosurgery Unit, Department of Head and Neck Surgery, Garibaldi Hospital, 95124 Catania, Italy"},{"name":"School of Medicine and Surgery, Kore University of Enna, 94100 Enna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentina","family":"Zagardo","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology, REM Radioterapia srl, 95125 Viagrande, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-3900-4419","authenticated-orcid":false,"given":"Zubayer","family":"Shams","sequence":"additional","affiliation":[{"name":"Brunel Medical School, Brunel University London, London UB8 3PH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2305-5544","authenticated-orcid":false,"given":"Gianluca","family":"Ferini","sequence":"additional","affiliation":[{"name":"School of Medicine and Surgery, Kore University of Enna, 94100 Enna, Italy"},{"name":"Department of Radiation Oncology, REM Radioterapia srl, 95125 Viagrande, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Salvatore","family":"Marrone","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Sant\u2019 Elia Hospital, 93100 Caltanissetta, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eliana","family":"Giurato","sequence":"additional","affiliation":[{"name":"Anatomic Pathology Unit, Garibaldi Hospital, 95124 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Graziano","sequence":"additional","affiliation":[{"name":"Neurosurgery Unit, Department of Head and Neck Surgery, Garibaldi Hospital, 95124 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giancarlo","family":"Ponzo","sequence":"additional","affiliation":[{"name":"Neurosurgery Unit, Department of Head and Neck Surgery, Garibaldi Hospital, 95124 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Massimiliano","family":"Giuffrida","sequence":"additional","affiliation":[{"name":"Neurosurgery Unit, Department of Head and Neck Surgery, Garibaldi Hospital, 95124 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Massimo","family":"Furnari","sequence":"additional","affiliation":[{"name":"Neurosurgery Unit, Department of Head and Neck Surgery, Garibaldi Hospital, 95124 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1573-431X","authenticated-orcid":false,"given":"Giuseppe Emmanuele","family":"Umana","sequence":"additional","affiliation":[{"name":"School of Medicine and Surgery, Kore University of Enna, 94100 Enna, Italy"},{"name":"Department of Neurosurgery, Trauma and Gamma Knife Center, Cannizzaro Hospital, 95126 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanni Federico","family":"Nicoletti","sequence":"additional","affiliation":[{"name":"Neurosurgery Unit, Department of Head and Neck Surgery, Garibaldi Hospital, 95124 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,4,29]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"817","DOI":"10.1097\/00000478-198910000-00001","article-title":"Ossifying fibromyxoid tumor of soft parts. 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