Desmoid fibromatosis of submandibular region.

We report a case of desmoid tumor in the submandibular region in a 32-year-old male who presented with right submandibular swelling postextraction of right lower wisdom tooth. Excision biopsy was carried out initially following inconclusive fine needle aspiration and discussion at multidisciplinary meeting. The tumor recurred 4 months following initial excisional biopsy necessitating a more radical secondary approach involving segmental mandibulectomy. Intraoperatively we also noted that the tumor was originating from the site of previous wisdom tooth extraction, raising the question of surgical trauma as precursor of desmoid tumor. We achieved a negative resection margin and a complete remission for 24 months.

Butel-Simoes GI, Spigelman AD.

Xhaja X, Church J.

Desmoid-type fibromatosis (desmoid) is a locally aggressive (myo)fibroblastic lesion. It represents one of the more common fibrous tumours in children and adolescents. The head and neck region is more often involved than in adults. Our study provides evidence of genetic similarities in desmoid and desmoplastic fibroma. Additionally, we expanded the spectrum of mutations in CTNNB1 with one novel desmoid mutation.

Sarcomas are rare and heterogeneous with many subtypes explaining the high level of diagnostic difficulty with frequent important therapeutic consequences. In 2009, a national network of pathologists has been set up with the main objective to perform a systematic histological review of every new sarcoma, gastro-intestinal stromal tumor (GIST) and desmoid tumor. We describe the network organization and report the results of the first two years of activity.

The authors present a rare case of a hip contracture in a 28-year-old active man who presented with a functionally disabling hip flexion contracture of 20° with further flexion possible up to 130°, a 45° abduction contracture, and a 20° external rotation contracture with further rotation possible up to 40° with a bony hard swelling in the left gluteal region. Opposite hip and spine examinations were normal. The goals of treatment were to establish the causality between the 2 and to diagnose the etiology of the gluteal mass. Radiographs were normal, with only a pelvic obliquity evident. Magnetic resonance imaging revealed an extra-articular mass abutting the iliac blade. Histopathology confirmed the mass to be a desmoid tumor in the left gluteal region. A wide surgical excision of the mass was performed with negative margins; no postoperative radiotherapy was administered. After rigorous physiotherapy, the hip deformity disappeared at 6 months and there was no evidence of recurrence at 2.5-year follow-up, with the patient able to sit cross-legged and squat.