541 Post operative management of aggressive fibromatosis in childhood

Summary Aggressive fibromatosis (also known as desmoid tumors) arise from musculo-aponeurotic tissues and are histologically benign. They are however locally aggressive. Local relapse occurs in 20 to 100% of treated patients and is specially frequent following incomplete resection. We report 9 cases of children treated at Leon Berard Center between 1979–1991 by surgery and post operative treatment. Patients and methods Between 1979 and 1991, 9 children (7 male, 2 female) with aggressive fibromatosis were treated at Leon Berard Center. Ages ranged from 24 to 204 months. Five were seen at first relapse and 4 were treated in first intent. All specimens were reviewed by the Department of Pathology, (histological confirmation of aggressive fibromatosis was confirmed in all cases). Involved sites of disease include: limbs (4), supraclavicular (2), face (2) and sacrum (1). Initial surgery consisted of wide excision in 7 patients with a macroscopic residual disease, and a microscopic residual disease in 2 cases. Despite partial surgery in 5/9 patients who were treated at other institutions, no further post-operative treatment was proposed and these patients were referred at Centre Leon Berard with local recurrences following first surgical resection. All of them underwent a record surgery at time of relapse. Four patients were referred for initial diagnosis of desmoid tumors and had an incomplete surgery. Four children were treated with postoperative radiation, 2 with radiation and chemotherapy, 1 child with radiation therapy, chemotherapy and endocrine therapy and 1 with endocrine therapy alone. Results: Local control Six patients relapsed within 4 to 12 months from surgery. Three were controlled. Among the controlled patients, 1 received radiation therapy alone (45 Gy), the second received chemora-diation (Ifosfamide, Vincristin, Adriamycin + 45 Gy); the third received Tamoxifen (20 mg/m 2 ). Second surgery was performed on the 6 patients who failed locally: macroscopic complete resection was performed but with positive margin in 3 cases. Gross residual disease was present in two cases, the last one achieved microscopically complete resection. No additional treatment was administered to the child who underwent a complete resection; he is alive at 10 years follow up. Adjuvant treatment was delivered to 5 patients: radiation therapy alone (2 pts); chemoradia-tion and TAM (2); chemotherapy alone (1). Survival All but one patients are still alive with a 93 months median follow up. Seven of 9 children are free of recurrence at last review. Nevertheless, one is still alive but with active disease. The last one died of disease 28 months after the initial surgery by locally uncontrolled tumor. Conclusion Primary treatment is surgical resection and should be as complete as possible. This is difficult to achieve. No patient in our study was completely resected. Most of children relapsed after the initial surgery and patients may died as a consequence of locally uncontrolled tumor. Theses findings confirm that fibromatosis are aggressive tumor which warrant aggressive therapy. Local control may be achieved with adjuvant ″aggressive″ treatment such as radiotherapy.