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

TL;DR

This paper reports a rare case of ameloblastic fibrosarcoma in an elderly woman's maxilla, which is unusual as it typically affects younger people and the mandible.

Contribution

The novelty lies in documenting an atypical AFS case in an elderly female arising from the maxilla, with detailed clinical and pathological findings.

Findings

AFS was diagnosed in a 66-year-old woman with a maxillary tumor, confirmed through histology and immunohistochemistry.

The patient underwent surgery and radiotherapy with no recurrence observed during a six-month follow-up.

Immunohistochemical markers showed positivity for Vimentin and SMA, but negativity for PCK and CD34.

Abstract

Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumor, commonly occurring in young adults 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.

A 66-year-old woman was admitted with a two-week history of a lump in her left upper molar. CT scans suggested a cyst in the maxillary bone. An incisional biopsy revealed a spindle cell neoplasm. MRI showed abnormalities in the left maxilla, indicating a possible tumorous lesion. The patient underwent a subtotal maxillectomy, wide tumor excision, intraoral epithelial flap transplantation, and dental extraction. Histology identified atypical tumor cells with visible mitotic figures. Immunohistochemistry showed negative for PCK and CD34 expression, but positive for Vimentin and SMA expression. The Ki-67 proliferation index ranged from 30 to 50%. These findings suggested a potentially malignant soft tissue tumor in the left maxilla, leaning towards a diagnosis of AFS. The patient received postoperative radiotherapy. There was no recurrence during the six-month follow-up.

Based on repeated pathological evidence, we report a rare case of an elderly female with AFS originating from the maxillary bone. Surgery and postoperative radiotherapy resulted in a favorable outcome.

The online version contains supplementary material available at 10.1186/s12903-024-04509-x.

Figures