Radiotherapy and conservative surgery in the management of musculo-aponeurotic fibromatosis

Fifty-four patients with musculo-aponeurotic fibromatosis treated with surgery, surgery and planned post-operative radiotherapy, or radiotherapy alone between 1936 and 1982 have been retrospectively reviewed. Twenty-seven patients had a previous excision before definitive treatment. All patients in whom surgery was known to be incomplete and who had no further treatment relapsed. Nine patients had a complete surgical excision alone and 1 relapsed. Twenty-nine patients were treated with surgery and post-operative radiotherapy and 7 relapsed. Relapse was associated with small field size, orthovoltage irradiation, and doses less than 50 Gy. Radiotherapy was effective in preventing relapse in 6 of 8 cases incompletely excised and in all of these cases the total dose was more than 50 Gy. In 13 assessable patients with clinically evident disease, 14 fields were treated with radiotherapy. Complete response was achieved in 9 fields (although one subsequently relapsed and 2 had a marginal relapse), partial response in 4, and disease stasis in one. Complete resolution took up to 21 months and total doses ranged from 35.2 Gy to 64 Gy. Radiotherapy is indicated in cases of incomplete excision and inoperable disease. Doses should be radical and fields should be sufficiently generous to encompass the anatomical limits of the infiltrated tissues.