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Official Journal of the Italian Society of Maxillofacial Surgery
Frequency: 3 issues
Online ISSN 1827-1901
Maremonti P., Califano L. *, Mangone G. M. *, Friscia M. *, Longo F. *, Insabato L. **
From the Operative Unit Maxillofacial Surgery, Bellaria Hospital of Bologna, Italy
* Maxillofacial Surgery Department and ** Department of Pathology, “Federico II” University of Naples, Italy
Desmoid tumour of the soft tissues first reported by MacFarlane in 1932, occurs most frequently in the abdominal cavity, but could also be located in the extremities. Desmoplastic fibroma is one of the rarest of bone tumours, it can be considered as the intraosseous counterpart of the much more common desmoid tumour of soft tissue, with only about 150 reported cases. The desmoplastic fibroma has been reported in different bones, the mandible is the most common site of disease, followed by the metaphysis of the femur, tibia or humerus. The aetiology is unknown, although trauma, endocrine and genetic factors have been suggested. The jaws are affected more frequently between 10 to 45 years of age with a mean of 17.2 years and a male to female ratio of 1.5 to 1. Symptoms are usually related to the expansion of the tumour; pain tenderness or paraesthesia are uncommon clinical findings. Pathological fractures have been reported in association with lesions of the long bones. Radiographically the lesions appear as well delineated radiolucencies with unilocular or multilocular aspect. The radiographic differential diagnosis often includes ameloblastoma, odontogenous myxoma, central haemangioma, aneurysmal bone cyst, chondromyxoid fibroma and eosinophilic granuloma. Macroscopically, desmoplastic tumours appear as a firm, rubbery, white non-encapsulated mass. Local recurrence is obviously strongly correlated with positive surgical margins. The authors report a case of a 45-year old white male with a painless lesion of the left mandibular angle. Resection of the left mandibular angle through a cervical approach was performed under general anaesthesia without reconstruction. The patient has been followed-up for 12 months with no evidence of recurrence. We are currently planning to perform secondary reconstruction.