Mandibular desmoid fibromatosis in Gardner Syndrome: A rare case report
Objectives: Gardner syndrome (GS) is a rare autosomal-dominant genetic disorder characterized by multiple colorectal adenomatous polyps and extraintestinal lesions, such as multiple osteomas of the bony face.The aim of this presentation is to describe the surgical approach of these maxillofacial localisations.
Methods: We report the case of a 24-year-old male patient with a family history of GS .He complained about swelling and pain in the region of the right mandibular angle . Radiographic findings revealed multiple osteomas in both sides of the mandible, impacted teeth, and odontomas . The Computed tomographic (CT) and MRI images showed a malignant osteolytic lesion of the right mandibular angle with an agressive periostal reaction . A gastrointestinal endoscopic examination was performed searching for intestinal polyps.
Results: The biopsy revealed a juvenile desmoid fibromatosis of the mandible .We performed a right segmental mandibulectomy with a submandibulectomy and bony osteosynthesis with a reconstruction plate.The gastrointestinal endoscopic examination didn’t reveal any intestinal polyps. The short-term esthetic results were excellent and judged satisfactory by the patient. A close radiological surveillance with MRI should be done to detect recurrence( which didnt occur during one year)
Conclusions: Desmoid tumours of the mandible are not usual in GS and should be treated completely . A close check up on these patients is recommended for intestinal polyposis malignant transformation.