COMBINATION CHEMOTHERAPY IN ADULT DESMOID TUMORS

Background. Desmoid tumors are locally aggressive tumors, with no meta static potential, that generally are amenable to local treatments, suc h as surgery and radiation therapy. Systemic therapy is considered for selected cases that are not amenable to local treatment. Methods. The authors reviewed their experience with chemotherapy in desmoid tumors . A patient population was identified through a search of the data bas e maintained by the Department of Patient Studies. Results. Between ja nuary 1971 and December 1991, 180 patients with a histologically confi rmed diagnosis of desmoid tumor were seen at the authors' institution. Twelve patients (8 male and 4 female patients; age range, 16-66 years ; median age, 29 years) received chemotherapy. Eleven patients receive d doxorubicin (60-90 mg/m2) plus dacarbazine (750-1000 mg/m2)-based re gimens for a median of 5 cycles (2-10 cycles). Six of the nine patient s who could be evaluated for response had an objective response (two c omplete responses and four partial responses), one patient had a minor response, and two patients had stable disease. Two other patients tre ated in the early 1970s could not be evaluated objectively because of lack of modern imaging; however, they were reported to have ''response s'' that enabled resection of axillary and pelvic disease. All four pa tients with Gardner syndrome experienced disease response. One of thes e four patients had a complete response twice with doxorubicin-based c hemotherapy and eventually died with an ejection fraction of 0.22. Fiv e patients are alive with no evidence of disease (NED), four are alive with disease, and two are lost to follow-up after having an NED statu s at their last visit. Conclusion. The authors conclude that desmoid t umors in adults are responsive to chemotherapy, and such treatment sho uld be considered before embarking on radical treatment to avoid obvio us functional consequences and delayed complications.