Nicolas Penel1, Axel Le Cesne2, Sylvie Bonvalot3, Antoine Giraud4, Emmanuelle Bompas5, Maria Rios6, Sébastien Salas7, Nicolas Isambert8, Pascaline Boudou-Rouquette9, Charles Honore2, Antoine Italiano10, Isabelle Ray-Coquard11, Sophie Piperno-Neumann12, François Gouin13, François Bertucci14, Thomas Ryckewaert15, Jean-Emmanuel Kurtz16, Françoise Ducimetiere11, Jean-Michel Coindre17, Jean-Yves Blay11. 1. Medical Oncology Department, Centre Oscar Lambret, Lille, France. Electronic address: n-penel@o-lambret.fr. 2. Department of Medical Oncology & Surgery, Institut Gustave Roussy, Villejuif, France. 3. Department of Surgery, Curie Institute, Paris, France. 4. Clinical and Epidemiological Research Unit, Institut Bergonie, Inserm CIC1401, Bordeaux, France. 5. Medical Oncology Department, René Gauducheau, Saint-Herblain, France. 6. Medical Oncology Department, Centre Alexis Vautrin, Nancy, France. 7. Medical Oncology Department, CH La Timone, Marseille, France. 8. Medical Oncology Department, Centre GF Leclerq, Dijon, France. 9. Department of Medical Oncology, CERIA, Paris Descartes University, AP-HP, Cochin Hospital, Paris, France. 10. Medical Oncology Department, Bergonié Institute, Bordeaux, France. 11. Centre Léon Bérard Lyon and Université Claude Bernard Lyon I, Lyon, France. 12. Department of Medical Oncology, Institut Curie, Paris, France. 13. CHU Hôtel-Dieu, Nantes, France; INSERM UI957, Nantes, France. 14. Medical Oncology Department, Institut Paoli-Calmette, Marseille, France. 15. Medical Oncology Department, Centre Oscar Lambret, Lille, France. 16. Department of Medical Oncology and Hematology, Hôpitaux Civils Universitaires Strasbourg, Strasbourg, France. 17. Department of Pathology, Institut Bergonié, Bordeaux, France.
Abstract
PURPOSE: The outcome of desmoid-type fibromatosis (DTF) is unpredictable. Currently, a wait-and-see approach tends to replace large en bloc resection as the first therapeutic approach. Nevertheless, there are no validated factors to guide the treatment choice. METHOD: We conducted a prospective study of 771 confirmed cases of DTF. We analysed event-free survival (EFS) based on the occurrence of relapse after surgery, progressive disease during the wait-and-see approach, or change in therapeutic strategy. Identification of prognostic factors was performed using classical methods (log-rank test and Cox model). RESULTS: Overall, the 2-year EFS was 56%; this value did not differ between patients undergoing an operation and those managed by the wait-and-see approach (53% versus 58%, p = 0.415). In univariate analysis, two prognostic factors significantly influenced the outcome: the nature of diagnostic sampling (p = 0.466) and primary location (p = 0.0001). The 2-year EFS was only 32% after open biopsy. The 2-year EFS was 66% for favourable locations (abdominal wall, intra-abdominal, breast, digestive viscera and lower limb) and 41% for unfavourable locations. Among patients with favourable locations, the 2-year EFS was similar in patients treated by both surgery (70%) and the wait-and-see approach (63%; p = 0.413). Among patients with unfavourable locations, the 2-year EFS was significantly enhanced in patients initially managed with the wait-and-see approach (52%) compared with those who underwent initial surgery (25%; p = 0.001). CONCLUSION: The location of DTF is a major prognostic factor for EFS. If these findings are confirmed by independent analysis, personalised management of DTF must consider this easily obtained parameter.
PURPOSE: The outcome of desmoid-type fibromatosis (DTF) is unpredictable. Currently, a wait-and-see approach tends to replace large en bloc resection as the first therapeutic approach. Nevertheless, there are no validated factors to guide the treatment choice. METHOD: We conducted a prospective study of 771 confirmed cases of DTF. We analysed event-free survival (EFS) based on the occurrence of relapse after surgery, progressive disease during the wait-and-see approach, or change in therapeutic strategy. Identification of prognostic factors was performed using classical methods (log-rank test and Cox model). RESULTS: Overall, the 2-year EFS was 56%; this value did not differ between patients undergoing an operation and those managed by the wait-and-see approach (53% versus 58%, p = 0.415). In univariate analysis, two prognostic factors significantly influenced the outcome: the nature of diagnostic sampling (p = 0.466) and primary location (p = 0.0001). The 2-year EFS was only 32% after open biopsy. The 2-year EFS was 66% for favourable locations (abdominal wall, intra-abdominal, breast, digestive viscera and lower limb) and 41% for unfavourable locations. Among patients with favourable locations, the 2-year EFS was similar in patients treated by both surgery (70%) and the wait-and-see approach (63%; p = 0.413). Among patients with unfavourable locations, the 2-year EFS was significantly enhanced in patients initially managed with the wait-and-see approach (52%) compared with those who underwent initial surgery (25%; p = 0.001). CONCLUSION: The location of DTF is a major prognostic factor for EFS. If these findings are confirmed by independent analysis, personalised management of DTF must consider this easily obtained parameter.
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