D Garbay1, A Le Cesne2, N Penel3, C Chevreau4, P Marec-Berard5, J-Y Blay6, M Debled1, N Isambert7, A Thyss8, E Bompas9, O Collard10, S Salas11, J-M Coindre12, B Bui1, A Italiano13. 1. Department of Medical Oncology, Institut Bergonie, Bordeaux. 2. Department of Medicine, Institut Gustave Roussy, Villejuif. 3. Department of Medical Oncology, Centre Oscar Lambret, Lille. 4. Department of Medical Oncology, Institut Claudius Regaud, Toulouse. 5. Institut d'hématologie et d'oncologie pédiatrique, Hospices civils de Lyon et Centre Léon Bérard, Lyon. 6. Department of Medical Oncology, Centre Léon Bérard, Lyon. 7. Department of Medical Oncology, Centre Georges-François Leclerc, Dijon. 8. Department of Medical Oncology, Centre Antoine-Lacassagne, Nice. 9. Department of Medical Oncology, Centre René Gauducheau, Nantes. 10. Department of Medical Oncology, Institut de Cancérologie de la Loire, Saint-Priest-en-Jarez. 11. Department of Medical Oncology, Hôpital La Timone, Marseille. 12. Department of Pathology, Institut Bergonie, Bordeaux, France. 13. Department of Medical Oncology, Institut Bergonie, Bordeaux. Electronic address: italiano@bergonie.org.
Abstract
BACKGROUND: Data regarding the role of chemotherapy (CT) in patients with recurrent and/or unresectable desmoid tumors (DTs) are scarce. PATIENTS AND METHODS: Records of patients with DT who were treated with CT in centers from the French Sarcoma Group were reviewed. RESULTS: Sixty-two patients entered the study. The two most common locations were extremities (35.5%) and internal trunk (32.5%). Twelve patients (19.5%) were diagnosed with Gardner syndrome. Thirty-seven patients (54.7%) received previously one or more lines of systemic therapies (nonsteroidal anti-inflammatory drugs: 43.5%, antiestrogens: 43.5% and imatinib: 30.5%). Combination CT was delivered in 44 cases (71%) and single agent in 18 patients (29%), respectively. Thirteen patients (21%) received an anthracycline-containing regimen. The most frequent nonanthracycline regimen was the methotrexate-vinblastine combination (n=27). Complete response, partial response, stable disease and progressive disease were observed in 1 (1.6%), 12 (19.4%), 37 (59.6%) and 12 (19.4%) patients, respectively. The response rate was higher with anthracycline-containing regimens: 54% versus 12%, P=0.0011. Median progression-free survival (PFS) was 40.8 months. The sole factor associated with improved PFS was the nonlimb location: 12.1 months (95% confidence interval 5.6-18.7) versus not reached, P=0.03. CONCLUSIONS: CT has significant activity in DT. Anthracycline-containing regimens appear to be associated with a higher response rate.
BACKGROUND: Data regarding the role of chemotherapy (CT) in patients with recurrent and/or unresectable desmoid tumors (DTs) are scarce. PATIENTS AND METHODS: Records of patients with DT who were treated with CT in centers from the French Sarcoma Group were reviewed. RESULTS: Sixty-two patients entered the study. The two most common locations were extremities (35.5%) and internal trunk (32.5%). Twelve patients (19.5%) were diagnosed with Gardner syndrome. Thirty-seven patients (54.7%) received previously one or more lines of systemic therapies (nonsteroidal anti-inflammatory drugs: 43.5%, antiestrogens: 43.5% and imatinib: 30.5%). Combination CT was delivered in 44 cases (71%) and single agent in 18 patients (29%), respectively. Thirteen patients (21%) received an anthracycline-containing regimen. The most frequent nonanthracycline regimen was the methotrexate-vinblastine combination (n=27). Complete response, partial response, stable disease and progressive disease were observed in 1 (1.6%), 12 (19.4%), 37 (59.6%) and 12 (19.4%) patients, respectively. The response rate was higher with anthracycline-containing regimens: 54% versus 12%, P=0.0011. Median progression-free survival (PFS) was 40.8 months. The sole factor associated with improved PFS was the nonlimb location: 12.1 months (95% confidence interval 5.6-18.7) versus not reached, P=0.03. CONCLUSIONS: CT has significant activity in DT. Anthracycline-containing regimens appear to be associated with a higher response rate.
Authors: Jae-Cheol Jo; Yong Sang Hong; Kyu-Pyo Kim; Jae-Lyun Lee; Jeeyun Lee; Young Suk Park; Sun Young Kim; Jin-Sook Ryu; Jong-Seok Lee; Tae Won Kim Journal: Invest New Drugs Date: 2014-01-16 Impact factor: 3.850