Desmoplastic ameloblastoma in the maxilla: a case report and review of literature.

Desmoplastic ameloblastoma (DA) is a rare variant of ameloblastoma (AM). The location of this lesion, its histology and radiological features differ from those of conventional AM. We report a case of DA in the canine / premolar region of the left maxilla of a 32-year-old woman and present a brief review of the literature. Radiographically, it had a mixed radiolucent / radiopaque appearance with ill-defined margins. Histologically, the tumor was characterized by extensive stromal desmoplasia and small tumor islands of odontogenic epithelium in the stroma, along with a few areas of reactive bone formation. The tumor was treated by partial maxillectomy and the patient was disease free after 1 year.

Malignant peripheral nerve sheath tumor of the maxilla.

The term malignant peripheral nerve sheath tumor (MPNST) refers to most spindle cell sarcomas arising from nerve or neurofibroma or showing nerve sheath differentiation and has been recently adopted by WHO. Malignant tumors of the peripheral nerve sheath are most commonly seen in association with the neurofibroma or at the site of irradiation and may also occur de novo. Its diagnosis has been called "one of the most difficult and elusive diagnosis in soft tissues diseases". We present a rare case of MPNST in a 12-year-old girl, who presented with a swelling of left cheek region of 2 months duration. Intraorally, the swelling extended buccally and palatally from premolar to tuberosity region. Swelling was fixed and firm to hard in consistency. A clinical diagnosis of odontogenic sarcoma was made. It was surgically excised and the histological features were suggestive of malignant peripheral nerve sheath tumor of the maxilla.