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Giant Desmoid Tumor of the Chest Wall

      A 21-year-old man was referred to our hospital because of a right thoracic mass detected on a medical checkup. He was asymptomatic. There was no history of trauma or Gardner syndrome. Computed tomography revealed that the chest wall tumor involved the right first and second ribs (Figure 1A). The tumor occupied the right hemithorax and displaced the mediastinum to the left (Figure 1B). A combined clamshell incision and median sternotomy were performed. The tumor was resected en bloc with portions of the first rib, second rib, sternum, musculus pectoralis major, and clavicle. The chest wall was reconstructed with a 1-mm composite mesh (polypropylene/polytetrafluoroethylene). Mechanical ventilation was required for 2 weeks postoperatively because of respiratory failure. However, the patient recovered without any functional deficit. Histopathological examination confirmed a desmoid tumor measuring 23.5 × 18 × 11 cm and weighing 2800 g. The specimen showed proliferations of spindle-shaped cells with slight atypia in a collagenous stroma. The surgical margins were free of tumor (Figure 2).
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      FIGURE 1Computed tomography scans of the chest. A, Axial image. B, Coronal image.
      Desmoid tumors are relatively rare tumors derived from fascial or musculoaponeurotic structures. Pathologically they are benign but grow locally aggressive. If feasible, surgical resection of the tumor is the treatment of choice, but a high incidence of local recurrence has been reported.
      • Abbas AE
      • Deschamps C
      • Cassivi SD
      • et al.
      Chest wall desmoid tumors: results of surgical intervention.
      • Meyerson SL
      • D'Amico TA
      Intrathoracic desmoid tumor: brief report and review of literature.
      Reoperation and positive margins are associated with a high risk of local recurrence.
      • Abbas AE
      • Deschamps C
      • Cassivi SD
      • et al.
      Chest wall desmoid tumors: results of surgical intervention.
      Therefore, it is essential to achieve local control by wide resection with tumor-free margins on initial treatment.

      REFERENCES

        • Abbas AE
        • Deschamps C
        • Cassivi SD
        • et al.
        Chest wall desmoid tumors: results of surgical intervention.
        Ann Thorac Surg. 2004; 78: 1219-1223
        • Meyerson SL
        • D'Amico TA
        Intrathoracic desmoid tumor: brief report and review of literature.
        J Thorac Oncol. 2008; 3: 656-659