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Retroperitoneal Sarcomas (RPS)

Patterns of Care and Prognosis of Retroperitoneal Sarcomas in the Primary and Advanced Settings A Large Multicentric Retrospective Analysis from the French Sarcoma Group .

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Retroperitoneal Sarcomas (RPS)

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  1. Patterns of Care and Prognosis of Retroperitoneal Sarcomas in the Primary and Advanced Settings A Large Multicentric Retrospective Analysis from the French Sarcoma Group M. Toulmonde, E Stoeckle, S. Bonvalot, N. Isambert, E Bompas, N. Penel, V. Brouste, J-Y. Blay, S. Piperno-Neumann, C. Chevreau, P. Terrier, D. Ranchère-Vince, J-M. Coindre, B. Nguyen Bui, A. Italiano

  2. Retroperitoneal Sarcomas (RPS) • Rare and heterogeneous • Poor prognosis • Heterogeneous data • Controversies • Type of surgery • Role of radiotherapy • Diversity of presentations in advanced setting

  3. Objectives • Description of patterns of care • Prognostic factors • Subgroup analyses according to histological subtypes • I) On a Large homogeneous cohort of patients with completely resected primary RPS • II) In the advanced setting

  4. Patients and Methods • Prospective database of the French Sarcoma Group • Retrospective review of medical charts • Patients ≥ 18 years with primary RPS • Diagnosis between 01/01/1988 and 31/12/2008 • Referred to one of 12 participating French Comprehensive Cancer Centers • Systematic histological review by an expert pathologist member of the GSF-GETO • Statistical analyses according to standard methods (Log-rank, Cox model)

  5. Results 19 patients initial abdominal sarcomatosis 586 patients • 586 patients • Median follow-up = 6.5 years [5.9-7.1] 30 patients initial distant metastasis 537 patients M0 26 patients not operated 511 patients operated 110 patients R2 12 patients unknown resection status 389 patients R0/R1

  6. Characteristics at diagnosis DDLPS: Dedifferentiated Liposarcoma WDLPS: Well-Differentiated Liposarcomas LMS: Leiomyosarcomas US: Unclassified Sarcomas Other: Other subtypes LR: Locoregional

  7. Initial treatment modalities

  8. LR relapse-free Survival Probability 100 LRrelapse-free Survival 75 95% CI 50 25 0 0 1 2 3 4 5 6 7 8 9 10 11 Time (years) Number at risk 389 326 258 197 138 94 70 52 41 26 20 13 Evolution (389 patients R0/R1) 5-year LR relapse-free Survival = 46% [41-52]

  9. Probability 100 Overall Survival 95% CI 75 50 25 0 0 1 2 3 4 5 6 7 8 9 10 11 Time (years) Number at risk 389 370 339 294 233 169 121 96 80 58 45 38 Evolution (389 patients R0/R1) Overall Survival 5-year overall survival = 66% [61-71]

  10. Predictive factors for complete surgery (511 patients operated )

  11. Prognostic factors for LR relapse LR relapse-free survival according to surgeon’s expertise 100 Expert surgeon No expert surgeon 50 0 0 1 3 5 7 9 11 Time (years) LR relapse-free survival according to perioperative radiotherapy 100 Perioperative radiotherapy No perioperative radiotherapy 50 0 0 1 3 5 7 9 11 Time (years)

  12. Prognostic factors for abdominal sarcomatosis Sarcomatosis-free survival according to tumor rupture 100 No tumor rupture Tumor rupture 50 0 0 1 3 5 7 9 11 Time (years)

  13. Prognostic factors for distant metastasis Distant metastasis-free survival according to histology 100 50 0 0 1 3 5 7 9 11 Time (years) WDLPS DDLPS US LMS Others

  14. Prognostic factors for overall survival Overall survival according to grade 100 Overall survival according to tumor rupture 100 No tumor rupture Tumor rupture 50 50 0 0 1 3 5 7 9 11 Time (years) 0 0 1 3 5 7 9 11 Time (years) grade 1 grade 2 grade 3

  15. Sub-group analysis according to histology for LR relapse

  16. Sub-group analysis according to histology for OS

  17. Patterns of care in advanced stage 586 patients 299 patients palliative CT for advanced disease 50 patients palliative surgery • First line palliative CT - Monotherapy = 49% • Anthracycline-based = 75% • Median number of line = 2 (1-8) • 33% received ≥ 3 lines

  18. OS from first line of palliative CT Overall Survival 100 75 Overallsurvival 95% CI 50 25 0 0 1 2 3 4 5 6 7 8 9 10 Time (years) Numberatrisk 299 168 85 44 28 12 4 2 1 0 0

  19. OS from first line of palliative CT Overall survival according to histology 100 WDLPS LMS 75 US DDLPS 50 Other 25 0 0 1 3 5 7 9 Time (years)

  20. Prognostic Factors for overall survival from first line of palliative CT Overall survival according to PS 100 PS = 0 PS =1 PS ≥ 2 50 0 0 1 3 5 7 9 Time (years) Overall survival according to grade 100 grade 1 grade 2 50 grade 3 Stage of the disease Palliative surgery • no difference 0 0 1 5 7 9 3 Time (years)

  21. Discussion • Predominance of DDLPS • Poor conformity to practice guidelines • Surgical expertise is a major prognostic factor • DDLPS  • WDLPS  LR involvement and OS • LMS  limits of surgery on OS • Need for histology-specific clinical trials - Radiotherapy and LR relapse - Grade and OS

  22. Acknowledgements • P. Terrier • D. Ranchère-Vince • A. Lecesne • S. Bonvalot • J-Y. Blay • S. Piperno-Neumann • C. Chevreau • J-O. Bay • A. Thyss • V. Brouste • J-B. Courrèges • E. Stoeckle • G. Kantor • A. Neuville • J-M. Coindre • B. Nguyen Bui • A. Italiano • O. Riou • N. Isambert • E Bompas • N. Penel • C. Delcambre-Lair • F. Duee • E. Saada • M. Jean-Denis

  23. Survivalsaccording to histology

  24. Sub-group analysis according to histology for overall survival after first line palliative CT

  25. LR relapse- free Survival (Probability) 100 LR-free Survival 75 95% CI 50 25 0 0 1 2 3 4 5 6 7 8 9 10 11 Time (years) Number at risk 389 326 258 197 138 94 70 52 41 26 20 13

  26. OverallSurvival according to grade 100 50 0 0 1 5 7 9 3 Time (years) Number at risk grade_pal = 1 46 30 16 10 7 3 1 1 1 0 0 grade 1 grade_pal = 2 125 75 46 21 14 4 1 0 0 0 0 grade 2 grade_pal = 3 111 50 16 9 4 3 2 1 0 0 0 grade 3 grade_pal = 1 grade_pal = 2 grade_pal = 3

  27. OverallSurvivalaccording to PS 100 PS = 0 75 PS =1 PS ≥ 2 50 25 0 0 1 3 5 7 9 Time (years) Number at risk ps_pal_recup_2c = 0 150 98 59 31 22 9 4 2 1 0 0 ps_pal_recup_2c = 1 97 47 22 10 4 1 0 0 0 0 0 ps_pal_recup_2c = 2 ou 3 28 8 1 0 0 0 0 0 0 0 0

  28. Sub-group analysis according to histology

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