Background: Malignant fibrous histiocytoma (MFH) is an aggressive, high-grade tumor with poor prognosis. It is the most common soft tissue sarcoma in adults, usually occurring retroperitoneally in extremities. MFH of the head and neck is rare. Cases with primary involvement of only the oral soft tissues have been reported; a few occurred in the gingiva. Its clinical features include a painless enlarging mass, which may or may not be ulcerated. Methods: A 20-year-old female patient presented with a non-healing necrotic ulceration of 4 months' duration on the maxillary anterior labial gingiva. Based on her history and agricultural background, a provisional diagnosis of chronic fungal and chronic bacterial infection was given. The patient was placed on a topical antifungal in combination with antibiotic therapy for 10 days. Because the lesion failed to respond to the initial therapy, an incisional biopsy was performed. The lesion was diagnosed as MFH based on clinical, histopathologic, and immunohistochemical findings. Results: The patient was referred to the Department of Oncology and Oral and Maxillofacial Surgery, Manipal College of Dental Sciences, Manipal, for further evaluation and treatment; the lesion was treated by resection of the premaxilla, and a splint was placed. She was followed up for 3 months with improvement in her condition. Conclusions: Because MFH is rare in the oral cavity, misdiagnosis may not be unusual based on the initial clinical examination. Any suspicious lesion that is unresponsive to treatment must be subjected to histopathologic evaluation to arrive at a final diagnosis.
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