Aggressive fibromatosis: optimisation of local management with a retrospective failure analysis

The records of 40 consecutive patients treated at the Princess Margaret Hospital (PMH) between 1979 and 1988 for aggressive fibromatosis were reviewed. The median follow-up was 86 months (range 21-167 months). All surgery was performed at the referring hospitals. Thirty-six underwent an attempt at excision, four were biopsied. Thirty-one had no overt disease after surgery and 26 of these received adjuvant irradiation. Eight were treated with radiotherapy alone, and another was treated with azathioprine and prednisone. Twenty-four (60%) presented with recurrent disease. The overall relapse free rate was 63% at 5 and 10 years. Combined surgery and irradiation had a higher relapse rate than irradiation alone (46% vs. 25%), and a high proportion of failures in the combined group were marginal failures (36%). Relapses following surgery alone were (1(5)) (20%), and chemotherapy(0(1)) . Tumour size greater than 8 cm predicted for relapse (p 0.002), but tumour site, status of surgical margins, and presence or absence of a history of relapse were not statistically significant. Twelve with subsequent treatment failure underwent successful salvage surgery, and (37(40)) (92%) were disease free at last follow-up. A functional assessment (modified Johnstone scale) revealed(11(24)) patients (46%) with poor functional outcomes (grade 2 or less) after all treatment compared with (6(24)) (25%) at referral. Ten of 11 (91%) with grade of 2 or less had a history of recurrence, and (4(5)) amputations were for treatment of a painful recurrence. Treatment planning in this study was hampered by inadequate information on tumour location since few patients had clinically apparent disease when seen by the radiation oncologist (only 35% of cases) and fewer had preoperative cross-sectional imaging available (12% of cases). Relapse was also associated with a poor functional outcome. Attaining high rates of local control with good functional outcomes requires a thorough pretreatment assessment of local disease and optimal selection, integration and delivery of the treatment modalities available. Combined surgery and irradiation should be considered and planned jointly for those patients at risk for relapse or with disease in sites where relapse would subsequently compromise function