# Ossifying Fibromyxoid Tumor of Soft Parts in the Head and Neck: A Systematic Review Addressing Surgical Management and Adjuvant Therapies

## TL;DR

This review examines the diagnosis and treatment of rare ossifying fibromyxoid tumors in the head and neck, emphasizing surgical removal and limited use of adjuvant therapies.

## Contribution

A systematic review of 99 cases providing insights into the clinical behavior, diagnosis, and treatment of OFMTs in the head and neck region.

## Key findings

- Most OFMTs in the head and neck are benign with low recurrence rates and no metastasis.
- Surgical excision with clear margins is the primary treatment, achieving good outcomes in most cases.
- Adjuvant therapies like radiotherapy are used in a small subset of cases with aggressive features or incomplete removal.

## Abstract

Ossifying fibromyxoid tumors (OFMTs) are rare soft-tissue tumors that can appear anywhere in the body but are found in the head and neck region in a minority of cases. These tumors often present as slow-growing, painless masses, making diagnosis challenging. This systematic review analyzed 99 cases to better understand how OFMTs in the head and neck are diagnosed and treated. Most tumors showed benign behavior, with low rates of recurrence and no recorded metastasis. Diagnosis relied on imaging, histological analysis, and immunohistochemistry, often showing features like fibromyxoid stroma and bone formation. The primary treatment was complete surgical removal, which usually resulted in excellent outcomes. Adjuvant therapies like radiation were used only in a few cases, mainly when surgery could not completely remove the tumor or when aggressive features were present. Understanding the behavior of OFMTs is important to avoid overtreatment while ensuring proper care. Future research should focus on molecular profiling to help predict the behavior of these rare tumors and guide personalized treatment and follow-up strategies.

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.