상악골에 발생한 악성 섬유성 조직구종 1례

Abstract

Purpose: Malignant fibrous histiocytoma (MFH) is mainly a soft tissue sarcoma containing fibroblast-like cells and histiocytic cells. MFH in bone accounts for 5% of all malignant bone tumors. MFH of the maxilla is extremely rare and difficult to diagnose due to its scarcity. Treatment mainstay is a complete surgical excision. Radiation therapy is also available when surgery alone is incomplete. Prognosis is not clear but can be devastating. Authors report one case of MFH developed in the maxilla. Methods: A 24-year-old man firstly diagnosed as fibrous dysplasia based on CT findings. Considering facial contour, partial excision was done. But pathology report confirmed malignant fibrous histiocytoma and secondary wide excision was done including zygoma and grossly all affected area. After surgery, radiation therapy was continued. Results: There are no evidence of tumor recurrence after clinical and radiological treatment.Conclusion: MFH of maxilla is very rare and this can leads to misdiagnosis in many clinicians. Surgeon should differentiate this disease from fibrous dysplasia and pathology and MRI are accurate methods for diagnosis of MFH.