PURPOSE: This retrospective study was conducted to assess outcome and to determine the prognostic factors in patients with a desmoid tumor treated with postoperative radiotherapy. MATERIALS AND METHODS: Twenty-seven patients with a desmoid tumor who were treated with postoperative radiotherapy between June 1984 and October 2005 were analyzed. There were 13 male and 14 female patients. The age of the patients ranged from 3 to 79 years (median age, 28 years). Tumors were located in an extra-abdominal area (21 cases), and in the abdominal walls (6 cases). The tumor size ranged from 2.5 to 25 cm (median size, 7.5 cm) in the largest linear dimension. Thirteen cases received radiotherapy after initial surgery, and 14 recurrent cases received radiotherapy after additional surgery. The total radiation dose given was 45~66 Gy (median dose, 59.4 Gy), and the fraction size was 1.8~2.0 Gy. RESULTS: The median follow-up period was 61 months (range, 12~203 months). Two patients developed local progression and six patients experienced local recurrence. The 5-year disease-free survival rate and the 5-year progression-free survival rate were 61% and 70%, respectively. Wide local excision was associated with better disease free survival with statistical significance (p=0.028). Radiotherapy after initial surgery (p=0.046) and a higher radiation dose of more than 60 Gy (p=0.049) were associated with better progression free survival with statistical significance. At the time of the last follow-up, the number of additional surgeries was higher in patients that received radiotherapy after reoperation (p<0.001). CONCLUSION: Radiotherapy after the initial operation improved local control and decreased the number of subsequent operations. Thus, postoperative radiotherapy after an initial operation is recommended in patients with a high risk of recurrence for a desmoid tumor.
Disease-Free Survival ; Female ; Fibromatosis, Aggressive* ; Follow-Up Studies ; Humans ; Male ; Radiotherapy ; Radiotherapy, Adjuvant* ; Recurrence ; Reoperation ; Retrospective Studies

Aggressive fibromatosis is a rare benign soft tissue tumor that is difficult to cure because of its infiltrative nature and high tendency to recur locally. The authors retrospectively analyzed 20 patients with histologically-confirmed fibromatosis. All patients underwent surgery with a wide or marginal margin. Five (25%) cases with histologically-negative margins had recurred. External beam radiotherapy was administered to patients whose margins were positive or who had local recurrence. However, out of concern for safety, radiotherapy was not given to two babies and a reproductive-aged woman. The average dose was 5,020 cGy. During the follow-up (mean 32.6 months), all the patients undergoing radiotherapy showed no evidence of local recurrence. A wide local excision has traditionally been the treatment of choice. However, postoperative radiotherapy could be an effective measure for preventing local recurrence in patients with a histologically-positive surgical margin and recurrence independent of any signs of relapse.
Adolescence ; Adult ; Aged ; Child ; Child, Preschool ; Combined Modality Therapy ; Female ; Fibromatosis, Aggressive/radiotherapy* ; Human ; Male ; Middle Age ; Neoplasm Recurrence, Local/radiotherapy

PURPOSE: To identify prognostic factors influencing progression-free survival (PFS) of aggressive fibromatosis (AF) after postoperative radiotherapy (PORT) and assess correlations between immunohistochemistry (IHC) features of β-catenin/smooth muscle actin (SMA) and PFS. MATERIALS AND METHODS: Records of 37 patients with AF treated by PORT from 1984 to 2015 were retrospectively reviewed. Fifteen patients underwent wide excision for AF and 22 patients received debulking operation. The median total dose of PORT was 59.4 Gy. IHC staining results of β-catenin and SMA were available for 11 and 12 patients, respectively. RESULTS: The median follow-up duration was 105.9 months. Five-year PFS rate was 70.9%. Tumor size or margin status was not related to PFS in univariate analysis (p = 0.197 and p = 0.716, respectively). Multivariate analysis showed that increased interval from surgery to PORT (>5.7 weeks) was a marginal risk factor for PFS (p = 0.054). Administration of PORT at the initial diagnosis resulted in significantly improved PFS compared to deferring PORT after recurrence (p = 0.045). Patient with both risk factors of deferring PORT after recurrence and interval from surgery to PORT >5.7 weeks had significantly lower 5-year PFS than patients without risk factor (34.1% vs. 100.0%; p = 0.012). Nuclear β-catenin intensity tended to inversely correlate with 5-year PFS, although it did not reach statistical significance (62.5% at low vs. 100.0% at high; p = 0.260). SMA intensity was not related to PFS (p = 0.700). CONCLUSION: PORT should be performed immediately after surgery irrespective of margin status or tumor size especially in recurrent case. Nuclear β-catenin staining intensity of IHC might correlate with local recurrence.
Actins ; beta Catenin ; Diagnosis ; Disease-Free Survival ; Fibromatosis, Aggressive ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Multivariate Analysis ; Radiotherapy ; Radiotherapy, Adjuvant ; Recurrence ; Retrospective Studies ; Risk Factors

Desmoid tumors are rare soft tissue neoplasms derived from fascial or musculoaponeurotic structures. These tumors are histologically benign but may behave aggressively at the local level with frequent recurrence after complete resection. Wide regional excision with negative pathologic margins is the treatment of choice for most desmoid tumors. A 36-year-old man was presented with a huge mass of 25 cm in diameter from right side of neck extended to ipsilateral chest wall. He was complaining discomfort during neck exercise and focal paresthesia on his right 2nd, 3rd, and 4th finger volar side without functional limitation. The lesion was evaluated preoperatively through MRI, angiographies and bone scan. It was originated from right scalenius medius muscle of neck extended to ipsilateral axilla and chest wall adhering to the brachial plexus. Tumor resection sparing brachial plexus was executed and postoperative radiotherapy was accompanied. After 9 months, there was no evidence of tumor recurrence and no major function limitation of right arm and hand.
Adult ; Angiography ; Arm ; Axilla ; Brachial Plexus ; Fibromatosis, Aggressive* ; Fingers ; Hand ; Humans ; Magnetic Resonance Imaging ; Neck* ; Paresthesia ; Radiotherapy ; Recurrence ; Soft Tissue Neoplasms ; Thoracic Wall* ; Thorax*