[Extra-abdominal desmoid tumors: 7-year outcome after surgical treatment in 12 patients]

The purpose of this study was to report the clinical course and outcome in 12 patients with extra-abdominal desmoid tumors.

This retrospective series included 12 patients with extra-abdominal desmoid tumors treated between 1987 and 1996. Female gender predominated (8/12) and age ranged from 4 months to 60 years. The tumors were identified in the thoracic wall (n=2), the upper limb (n=4), and the lower limb (n=6) with a bony localization in the upper femoral metaphysis. Magnetic resonance imaging was highly contributive to the search for extension. Surgical treatment was given in all cases. All patients were living at last follow-up.

Complete resection was achieved in three patients and incomplete resection in nine. Mean follow-up was seven years. There was one case of sarcomatous transformation and nine cases of recurrence. Mean time to recurrence was one year (range 6 months-4 years). Two tumors, in the forearm and the leg, did not recur. Secondary surgery was not attempted in four patients whose clinical situation has remained stable after recurrence. A second resection was performed for recurrence in four other patients who then remained recurrence-free to last follow-up. One tumor recurred three times after surgical resection. Functional outcome was good in nine patients.

The poorly limited nature of extra-abdominal desmoid tumors makes resection a difficult task, particularly when located in the girdles or the most proximal part of the limbs. Wide first-intention surgical resection is recommended but mutilation must be avoided due to the benign nature of the tumor, its slow progression, and the possibility of regression or stability after recurrence. Nevertheless, surgical treatment of recurrent tumors is difficult and often insufficient, compromising functional outcome. Complementary treatment (radiotherapy) has been advocated for better control tumor growth.