Abstract

A 60-year-old Japanese woman presented to the Department of Oral and Maxillofacial Surgery at Dokkyo University Hospital in 1982 with episodes of bleeding in the mouth. Clinical examination showed a 20 × 12 × 10 mm tumour in the right hard palate and no regional lymphadenopathy. It was excised under general anaesthesia and shown to be a pleomorphic adenoma. Follow-up was continued for six months but the patient was lost to follow-up until April 1994 when she presented to our hospital with a remarkable swelling of the face and repeated bleeding in the mouth. She was poorly nourished, her weight was 31 kg, and she could not walk by herself. She had a huge swelling of the right face roughly 170 × 200 × 140 mm in size, producing a severe form of hemifacial macrosomia with protrusion of the right buccal region and deviation of the nasal and lip regions to the left (Fig. 1). Conventional radiographs showed extensive resorption of the right maxillary bone and radio-opacities which indicated that the tumour was partially calcified. Magnetic resonance imaging (MRI) showed a tumour arising from the maxilla, almost entirely occupying the alveolar process and compressing the right maxillary sinus and the anterior aspect of the nasal cavity. The orbital floor seemed to be free of the lesion. The margins were relatively smooth and well-defined (Fig. 2). Incisional biopsy confirmed the diagnosis of carcinoma ex pleomorphic adenoma. In 1994 under general anaesthesia, through Webers and intraoral incisions, a flap was raised and the lesion was exposed. The mass extended to both maxillary bones, right maxillary sinus, right ethmoid sinus, right nasal fossa, right orbital floor, and right intratemporal fossa. There was no invasion in the right frontal sinus or cerebral region. The mass was almost surrounded by a capsule and was easily separated from the surrounding tissue. The tumour was completely Fig. 1 Photographs of the tumour: frontal view (left top), inferior view (right top), right lateral view (left bottom), and left lateral view (right bottom).

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