AGGRESSIVE FIBROMATOSIS - OPTIMIZATION OF LOCAL-MANAGEMENT WITH A RETROSPECTIVE FAILURE ANALYSIS

The records of 40 consecutive patients treated at the Princess Margare t Hospital (PMH) between 1979 and 1988 for aggressive fibromatosis wer e reviewed. The median follow-up was 86 months (range 21-167 months). All surgery was performed at the referring hospitals. Thirty-six under went an attempt at excision, four were biopsied. Thirty-one had no ove rt 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 rec urrent disease. The overall relapse free rate was 63% at 5 and 10 year s. Combined surgery and irradiation had a higher relapse rate than irr adiation alone (46% vs, 25%), and a high proportion of failures in the combined group were marginal failures (36%). Relapses following surge ry alone were 1/5 (20%), and chemotherapy 0/1. Tumour size greater tha n 8 cm predicted for relapse (p = 0.002), but tumour site, status of s urgical margins, and presence or absence of a history of relapse were not statistically significant. Twelve with subsequent treatment failur e underwent successful salvage surgery, and 37/40 (92%) were disease f ree at last follow-up. A functional assessment (modified Johnstone sca le) 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, a nd 4/5 amputations were for treatment of a painful recurrence, Treatme nt planning in this study was hampered by inadequate information on tu mour location since few patients had clinically apparent disease when seen by the radiation oncologist (only 35% of cases) and fewer had pre operative cross-sectional imaging available (12% of cases). Relapse wa s also associated with a poor functional outcome, Attaining high rates of local control with good functional outcomes requires a thorough pr etreatment assessment of local disease and optimal selection, integrat ion and delivery of the treatment modalities available. Combined surge ry and irradiation should be considered and planned jointly for those patients at risk for relapse or with disease in sites where relapse wo uld subsequently compromise function.