Ameloblastic fibrosarcoma of the maxilla arising in an old woman, a rare case report and literature review

Abstract Background Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumor, commonly occurring in adolescents and typically affecting the mandibular region. We report an exceptionally rare and highly atypical case of AFS in an elderly female patient originating from the maxillary bone. Case presentation: A 66-year-old woman was admitted to the hospital due to a left upper molar lump felt for two weeks. A CT scan suggested a maxillary bone cyst possibility.An incisional biopsy identified a spindle cell neoplasm within the soft tissues. Following an MRI, abnormalities were revealed in the left maxilla and its adjacent tissues, raising concerns about a tumorous lesion.The patient underwent subtotal maxillectomy, wide excision of the malignant tumor, intraoral epithelial flap transplantation, and dental extraction on June 15, 2023. Post-surgery histology exhibited atypical tumor cells arranged in bundles or woven patterns, with visible nuclear mitotic figures. Immunohistochemistry results indicated that tumor cells exhibited negative expression levels for PCK and CD34, while being positive for Vimentin and SMA. Additionally, the Ki-67 proliferation index ranged from 30–50% These findings suggested a potential malignant soft tissue tumor in the left maxilla, leaning towards AFS diagnosis. However, additional consultation was recommended due to the case's rarity, but the patient declined due to financial limitations. The patient received postoperative radiotherapy one month after surgery. She recovered well, with no recurrence recorded during a 6-month follow-up. Conclusion Based on repeated pathological evidence, we report a rare case of an elderly female with AFS originating from the maxillary bone. The patient underwent surgery and postoperative radiotherapy, resulting in a favorable treatment outcome.