Giant cell tumor of the mandible in a patient with Paget's disease

Giant cell tumor (GCT) is a rare complication of PD. Thepresent study reports a new case of GCT involving themandible and maxilla in a patient with PD.A 53-year-old man from Naples, Southern Italy, hadpolyostotic PD of bone since 1987. On admission, physicalexamination disclosed bilateral enlargement of the skull thaton radiology showed the typical “cotton wool” appearance.Laboratory investigations disclosed altered serum levels ofalkaline phosphatase (1380 IU/L; normal range, 30 to 135IU/L), whereas serum calcium levels were within normalrange, as were calcitonin and parathyroid hormone.In August 2001, he had a right scapular lesion that wassurgically removed and histologically diagnosed as GCT. InDecember 2001, he presented a rapidly enlarging mass of theleft mandible body. On radiology, the lesion presented as ahuge osteolytic lesion invading the surrounding soft tissues(Fig 1A). The surrounding mandibular bone showed radiologicfeatures indicating Pagetic involvement. The lesion was surgi-cally removed. Under general anesthesia, surgery was under-taken through an intraoral approach in the gingiva along theentire mandibular arch. The heavily bleeding tumor was iso-lated and excised, and the residual mandibular bone was sub-mitted to careful curettage. The left alveolar nerve, completelyinvolved by the tumor, was sacrificed. A local hematoma wasdrained on the fourth postoperative day. Despite radical surgi-cal treatment, the lesion recurred twice, in June 2002 and inApril 2003. In May 2003, the patient had a swelling of the rightorbital region. Radiology disclosed a large osteolytic lesioninvolving the ethmoid bone and extending to the orbital tissues.An incisional biopsy revealed a new localization of GCT, andthe lesion was surgically removed.At histology, all the lesions had the same appearance. Alow-power view disclosed multiple giant cells uniformlydistributed throughout the lesion (Fig 1B). Giant cells hadnumerous nuclei and lay in a background of oval to spindlemononuclear cells. Mitotic figures were abundant. No areasof hemorrhage or necrosis were seen, nor was osteoid ma-terial present. The lesion diffusely invaded the periman-dibular soft tissues. The histologic picture was identical tothat usually observed in GCT of long bones. The bonetissue surrounding the lesion showed the typical featuresof Paget’s disease withenlarged marrow spaces containinginflammatory infiltrate and giant cells (Fig 1C).