Background:Ossifying fibromyxoid tumors (OFMTs) are rare mesenchymal neoplasms with behaviors ranging from benign to malignant. Although most occur in the extremities and trunk, 9–13% 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.
背景：骨化性纤维黏液样肿瘤（OFMTs）是一种罕见的间叶源性肿瘤，其生物学行为可从良性至恶性。尽管多数发生于四肢和躯干，但仍有9%–13%的病例出现在头颈部，如口腔、头皮和颅盖骨。由于其罕见性及与其他肿瘤的组织学相似性，诊断颇具挑战。本综述综合分析了头颈部OFMTs的临床表现、诊断特征及治疗结局的相关证据，重点关注外科治疗及辅助治疗策略。 方法：根据PRISMA指南进行系统综述，检索PubMed/MEDLINE、Embase、Scopus和Web of Science数据库。纳入1989年至2024年间报道头颈部OFMTs且包含临床、组织病理学及治疗数据的研究。提取数据包括人口统计学特征、肿瘤特征、手术切缘、辅助治疗及预后情况。 结果：共纳入40项研究，涉及99例患者。患者年龄范围为3周至88岁（中位年龄47岁），男性居多（63.64%）。最常见的临床表现为缓慢生长、无痛性肿块。肿瘤最常见于颈部、口腔、头皮和颅盖骨。组织病理学显示70%的病例为有包膜的肿瘤，伴有纤维黏液样间质、梭形细胞及外周化生性骨组织边缘。免疫组化显示S-100、波形蛋白和SOX10阳性。手术切除是主要治疗方式（占28.28%的病例），复发率为9.09%，无转移病例。15.15%的病例接受了辅助治疗（主要为放疗）。 结论：头颈部OFMTs是罕见肿瘤，需要多学科协作诊疗。影像学、组织病理学和免疫组化是诊断的关键。手术切除并确保切缘阴性仍是主要治疗手段，且复发率较低。对于恶性或未完全切除的病例可能需要辅助治疗。未来需进一步研究以优化随访方案，并通过分子谱分析评估风险分层。
肿瘤(癌症)患者之家