Combination chemotherapy in adult desmoid tumors.

BACKGROUND: Desmoid tumors are locally aggressive tumors, with no metastatic potential, that generally are amenable to local treatments, such 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 base maintained by the Department of Patient Studies.
RESULTS: Between January 1971 and December 1991, 180 patients with a histologically confirmed 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 received doxorubicin (60-90 mg/m2) plus dacarbazine (750-1000 mg/m2)-based regimens for a median of 5 cycles (2-10 cycles). Six of the nine patients who could be evaluated for response had an objective response (two complete responses and four partial responses), one patient had a minor response, and two patients had stable disease. Two other patients treated in the early 1970s could not be evaluated objectively because of lack of modern imaging; however, they were reported to have "responses" that enabled resection of axillary and pelvic disease. All four patients with Gardner syndrome experienced disease response. One of these four patients had a complete response twice with doxorubicin-based chemotherapy and eventually died with an ejection fraction of 0.22. Five 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 status at their last visit.
CONCLUSION: The authors conclude that desmoid tumors in adults are responsive to chemotherapy, and such treatment should be considered before embarking on radical treatment to avoid obvious functional consequences and delayed complications.