C Honoré1, J B Delhorme2, E Nassif3, M Faron4, G Ferron5, E Bompas6, O Glehen7, A Italiano8, F Bertucci9, D Orbach10, M Pocard11, F Quenet12, J Y Blay13, S Carrere12, C Chevreau14, O Mir15, A Le Cesne3. 1. Department of Surgical Oncology, Gustave Roussy Cancer Campus, Villejuif, France. Electronic address: Charles.HONORE@gustaveroussy.fr. 2. Department of General and Digestive Surgery, Hautepierre Hospital, Strasbourg University, Strasbourg, France. 3. Department of Cancer Medicine, Gustave Roussy Cancer Campus, Villejuif, France. 4. Department of Surgical Oncology, Gustave Roussy Cancer Campus, Villejuif, France. 5. Department of Surgery, Claudius Régaud Institute (IUCT), Toulouse, France. 6. Department of Medical Oncology, West Cancer Institute (ICO), Nantes, France. 7. Department of Surgical Oncology, Lyon Civil Hospices, South Lyon University Hospital Center, Lyon, France. 8. Department of Medical Oncology, Bergonié Institute, Bordeaux, France. 9. Department of Medical Oncology, Paoli-Calmettes Institute, Aix-Marseille University, Marseille, France. 10. SIREDO Oncology Center (Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer), Institut Curie, PSL University, Paris, France. 11. Department of Digestive and Oncological Surgery, Lariboisière University Hospital, Paris, France. 12. Department of Surgical Oncology, Regional Cancer Institute of Montpellier (ICM), Montpellier, France. 13. Department of Medical Oncology, Leon Bérard Center, Lyon, France. 14. Department of Medical Oncology, Claudius Régaud Institute (IUCT), Toulouse, France. 15. Department of Ambulatory Care, Gustave Roussy Cancer Campus, Villejuif, France.
Abstract
BACKGROUND: Despite being associated with a very poor prognosis, long-term survivors across all series of Desmoplastic Small Round Cell Tumor (DSRCT) have been reported. AIM OF THE STUDY: To analyze patients 'characteristics associated with a prolonged survival after DSRCT diagnosis. METHODS: All consecutive patients treated for DSRCT in nine French expert centers between 1991 and 2018 were retrospectively analyzed. Patients with a follow-up of less than 2 years were excluded and cure defined as being disease-free at least 5 years. RESULTS: 100 pts were identified (median age 25 years, 89% male). 27 had distant metastases at diagnosis and 80 pts underwent upfront chemotherapy (CT). 71 pts were operated, 20 pts without prior CT). Surgery was macroscopically complete (CC0/1) in 50 pts. Hyperthermic intraperitoneal Chemotherapy (HIPEC) was administered during surgery in 15 pts 54 pts had postoperative CT and 26 pts had postoperative whole abdomino-pelvic RT (WAP-RT). After a median follow-up of 103 months (range 23-311), the median overall survival (OS) was 25 months. The 1- year, 3-year and 5-year OS rates were 90%, 35% and 4% respectively. 5 patients were considered cured after a median disease-free interval of 100 months (range 22-139). Predictive factors of cure were female sex (HR = 0.49, p = 0.014), median PCI<12 (HR = 0.32, p = 0.0004), MD Anderson stage I (HR = 0.25, p < 0.0001), CC0/1 (HR = 0.34, p < 0.0001), and WAP-RT (HR = 0.36, p = 0.00013). HIPEC did not statistically improve survival. CONCLUSION: Cure in DSRCT is possible in 5% of patients and is best achieved combining systemic chemotherapy, complete cytoreductive surgery and WAP-RT. Despite aggressive treatment, recurrence is common and targeted therapies are urgently needed.
BACKGROUND: Despite being associated with a very poor prognosis, long-term survivors across all series of Desmoplastic Small Round Cell Tumor (DSRCT) have been reported. AIM OF THE STUDY: To analyze patients 'characteristics associated with a prolonged survival after DSRCT diagnosis. METHODS: All consecutive patients treated for DSRCT in nine French expert centers between 1991 and 2018 were retrospectively analyzed. Patients with a follow-up of less than 2 years were excluded and cure defined as being disease-free at least 5 years. RESULTS: 100 pts were identified (median age 25 years, 89% male). 27 had distant metastases at diagnosis and 80 pts underwent upfront chemotherapy (CT). 71 pts were operated, 20 pts without prior CT). Surgery was macroscopically complete (CC0/1) in 50 pts. Hyperthermic intraperitoneal Chemotherapy (HIPEC) was administered during surgery in 15 pts 54 pts had postoperative CT and 26 pts had postoperative whole abdomino-pelvic RT (WAP-RT). After a median follow-up of 103 months (range 23-311), the median overall survival (OS) was 25 months. The 1- year, 3-year and 5-year OS rates were 90%, 35% and 4% respectively. 5 patients were considered cured after a median disease-free interval of 100 months (range 22-139). Predictive factors of cure were female sex (HR = 0.49, p = 0.014), median PCI<12 (HR = 0.32, p = 0.0004), MD Anderson stage I (HR = 0.25, p < 0.0001), CC0/1 (HR = 0.34, p < 0.0001), and WAP-RT (HR = 0.36, p = 0.00013). HIPEC did not statistically improve survival. CONCLUSION: Cure in DSRCT is possible in 5% of patients and is best achieved combining systemic chemotherapy, complete cytoreductive surgery and WAP-RT. Despite aggressive treatment, recurrence is common and targeted therapies are urgently needed.
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