Scalia Gianluca, Zagardo Valentina, Shams Zubayer, Ferini Gianluca, Marrone Salvatore, Giurato Eliana, Graziano Francesca, Ponzo Giancarlo, Giuffrida Massimiliano, Furnari Massimo, Umana Giuseppe Emmanuele, Nicoletti Giovanni Federico
Neurosurgery Unit, Department of Head and Neck Surgery, Garibaldi Hospital, 95124 Catania, Italy.
School of Medicine and Surgery, Kore University of Enna, 94100 Enna, Italy.
Cancers (Basel). 2025 Apr 29;17(9):1508. doi: 10.3390/cancers17091508.
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. 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. 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. 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.
骨化性纤维黏液样肿瘤（OFMTs）是一种罕见的间叶性肿瘤，其行为从良性到恶性不等。虽然大多数发生在四肢和躯干，但9%至13%发生在头颈部，如口腔、头皮和颅骨。由于其罕见性以及与其他肿瘤在组织学上的相似性，诊断具有挑战性。本综述综合了关于头颈部OFMTs的临床表现、诊断特征和治疗结果的证据，重点关注手术管理和辅助治疗。根据PRISMA指南进行了系统综述，在PubMed/MEDLINE、Embase、Scopus和科学网进行了检索。纳入了1989年至2024年报告头颈部OFMTs并带有临床、组织病理学和治疗数据的研究。提取的数据包括人口统计学、肿瘤特征、手术切缘、辅助治疗和结果。纳入了40项研究共99例患者。患者年龄从3周至88岁（中位数47岁），男性占优势（63.64%）。最常见的表现是生长缓慢、无痛性肿块。肿瘤最常发生在颈部、口腔、头皮和颅骨。组织病理学显示70%的病例为有包膜的肿瘤，伴有纤维黏液样基质、梭形细胞和周边化生骨。免疫组织化学显示S-100、波形蛋白和SOX10呈阳性。手术切除是主要治疗方法，28.28%的病例采用，复发率为9.09%，无转移。15.15%的病例采用辅助治疗，主要是放疗。头颈部OFMTs是需要多学科护理的罕见肿瘤。影像学、组织病理学和免疫组织化学是诊断的关键。切缘清晰的手术切除仍然是主要治疗方法，复发率低。恶性或切除不完全的病例可能需要辅助治疗。需要进一步研究以优化随访方案并评估分子谱以进行风险分层。
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