DESMOPLASTIC FIBROMA OF THE BONE - A REPORT OF 2 PATIENTS, REVIEW OF THE LITERATURE, AND THERAPEUTIC IMPLICATIONS

BACKGROUND. Desmoplastic fibroma (DF) is an extremely rare bone tumor. The recommendations for therapy are often based on limited personal e xperience, and the rate of local recurrence in the published cases is very high. Therefore, an analysis of treatment results of published ca ses was performed. Furthermore, DNA analysis of the tumors from two pa tients was also performed. METHODS. The clinical, radiologic, and hist ologic data of two patients with DF of the long bones are presented. D NA flow cytometry was performed on both DFs, three cases of abdominal fibromatosis, and three cases of extraabdominal fibromatosis. One hund red eighty-nine patients analyzed in the literature and our own 2 pati ents were evaluated with regard to epidemiologic, clinical, and histol ogic data, with particular emphasis on treatment results. RESULTS. DNA analysis of the locally infiltrating tumors revealed indices of proli feration between 21.5% and 24%, noticeably elevated values in comparis on with extraosseous desmoid tumors (8.04%). Magnetic resonance imagin g (MRI) was most valuable for imaging the intraosseous and extraosseou s extent of DF. The evaluation of 191 patients (189 from the Literatur e, 2 of the authors) showed the numbers of males and females to be equ ivalent, with a mean age of 23 years. DF has been reported in almost a ll bones, with a tendency to occur in the mandible and the long bones. Approximately 12% of patients presented with a pathologic fracture (2 0 of 161 patients). Infiltrative growth in the soft tissue was documen ted in 48% of patients. Three patients developed metastases after loca l recurrence. Analyzing the treatment results, the authors found a rec urrence rate of 55-72% after nonresection procedures, and 17% after re section. No recurrences are reported after resection with wide surgica l margins. The recurrence rate of tumors of the extremities was 55%, a nd 25% of these patients eventually required an amputation. CONCLUSION S. Considering the ''semimalignant'' character of this entity and the poor treatment results in patients with recurrent tumors, marginal or wide resection for primary treatment is recommended. The superior imag ing quality of MRI greatly facilitates preoperative planning. (C) 1996 American Cancer Society.