[Therapeutic options for aggressive fibromatosis]. / Therapieoptionen bei der aggressiven fibromatose.

INTRODUCTION: Aggressive fibromatosis, e.g., desmoid tumour, is a rare neoplasm of the connective tissue with local infiltrative growth. Because of the high recurrence rates and destruction of the surrounding tissue, these tumours are classified as semi-malignant. However, desmoid tumors tend not to metastasise. Arising from deep musculoaponeurotic structures, a monoclonal proliferation of fibroblasts occurs. Radical surgical treatment with tumour excision accompanied by radiotherapy is the current standard therapy that can be supplemented by pharmacological treatment in a few cases. PATIENTS AND METHOD: We report on 9 patients (5 males and 4 females) with surgical therapy for aggressive fibromatosis. The neoplasm was located on the extremities in 7 cases (4 x upper extremity, 3 x lower extremity), one tumour was situated in the chin and one in the rectus abdominis muscle. All patients were treated with radical tumour resection. Postoperatively, adjuvant radiotherapy was performed depending on the resection level of each patient. RESULTS: In 6 cases complete tumour resection (R0) was achieved. Following surgery, regular follow-ups with a physical examination and MR images were performed. In 3 cases a total tumour resection was impossible (R1 resection). The aggressive fibromatosis had infiltrated the pelvis in 2 cases implying a hemipelvectomy for R0 resection which was not practicable. Furthermore, diffuse locoregional infiltration of the chin region occurred in one case. In these cases, an adjuvant radiotherapy with 25 x 2 Gy was started postoperatively after the accomplished wound healing. Treatment with chemotherapeutic agents was not necessary. DISCUSSION: Aggressive fibromatosis is a semimalignant neoplasm of the connective tissue with an extremely high recurrence rate. Macroscopically, indistinct solid lesions are found with the destruction of muscles and other surrounding structures. Therapy of choice is the radical surgical resection. Radiotherapy is indicated for patients with non-resectable tumours. Pharmacological treatment should be considered for patients with unsuccessful local therapy.