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Marcus Tan , Murray F Brennan, Deborah Kuk ,

The I mportance of Histologic Subtype for Outcome Prediction in Primary Retroperitoneal Sarcoma : A Single-Institution Study of 675 Patients . Marcus Tan , Murray F Brennan, Deborah Kuk , Li- Xuan Qin, Narasimhan Agaram , Nicole Moraco , Aimée Crago , Samuel Singer

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Marcus Tan , Murray F Brennan, Deborah Kuk ,

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  1. The Importance of Histologic Subtype for Outcome Prediction in Primary Retroperitoneal Sarcoma: A Single-Institution Study of 675 Patients Marcus Tan, Murray F Brennan, Deborah Kuk, Li-XuanQin, NarasimhanAgaram, Nicole Moraco, AiméeCrago, Samuel Singer Memorial Sloan-Kettering Cancer Center Sarcoma Disease Management Program

  2. Histology & RP sarcoma Retroperitoneal sarcomas (RPS) • Complete resection is the only chance for cure • Role of radiation therapy and chemotherapy remain controversial • AJCC staging system of limited utility • Increasing understanding of differences in histologic types and subtypes • Need for histology-based models to better predict patterns of failure for individual patients

  3. Histology & RP sarcoma Aim and Hypothesis • Aim: To determine the prognostic significance of histologic type/subtype in a large series of patients with primary retroperitoneal sarcoma • Hypothesis: Each histologic type/subtype of retroperitoneal sarcoma is associated with distinct patterns of local and distant recurrence

  4. Histology & RP sarcoma Methods • Retrospective review of prospectively-maintained sarcoma database from July 1982 to July 2010 • 675 patients with primary, non-metastatic RPS (excluding desmoid& GIST) • Median follow-up for survivors 4.3 years • Survival calculated from time of initial operation

  5. Histology & RP sarcoma Tumor Characteristics

  6. Histology & RP sarcoma Operative Details *Subsequently excluded from analyses

  7. Histology & RP sarcoma Disease-Specific Death (DSD) by histologic type/subtype (n=184) n=632* p<0.0001 (n=227) (n=119) (n=32) (n=21) *Resected patients only

  8. Histology & RP sarcoma Multivariate analysis of DSD

  9. Histology & RP sarcoma Local Recurrence (LR) by histologic type/subtype n=574* p<0.0001 (n=173) (n=197) (n=113) (n=29) (n=18) *R0/R1 resections only

  10. Histology & RP sarcoma Multivariate analysis of LR

  11. Histology & RP sarcoma Local Recurrence Nomogram Low grade LMS MPNST DD LPS WD LPS High grade LMS SFT Concordance index 0.69

  12. Histology & RP sarcoma Distant Recurrence (DR) by histologic type/subtype (n=184) n=632* p<0.0001 (n=227) (n=119) (n=32) (n=21) *Resected patients only

  13. Histology & RP sarcoma Multivariate Analysis of DR

  14. Histology & RP sarcoma Distant Recurrence Nomogram Low grade LMS Other MPNST SFT WD LPS DD LPS High grade LMS Concordance index 0.72

  15. Histology & RP sarcoma Natural History by Histologic Type/Subtype Disease-Specific Death (n=184) Well-diff & myxoid liposarcoma (n=227) Dedifferentiated, RC & pleomorphic liposarcoma (n=119) High grade leiomyosarcoma (n=32) Solitary fibrous tumor (n=21) MPNST Local Recurrence Distant Recurrence

  16. Histology & RP sarcoma Natural History by Histologic Type/Subtype Disease-Specific Death (n=184) Well-diff & myxoid liposarcoma (n=227) Dedifferentiated, RC & pleomorphic liposarcoma (n=119) High grade leiomyosarcoma (n=32) Solitary fibrous tumor (n=21) MPNST Local Recurrence Distant Recurrence

  17. Histology & RP sarcoma Natural History by Histologic Type/Subtype Disease-Specific Death (n=184) Well-diff & myxoid liposarcoma (n=227) Dedifferentiated, RC & pleomorphic liposarcoma (n=119) High grade leiomyosarcoma (n=32) Solitary fibrous tumor (n=21) MPNST Local Recurrence Distant Recurrence

  18. Histology & RP sarcoma Natural History by Histologic Type/Subtype Disease-Specific Death (n=184) Well-diff & myxoid liposarcoma (n=227) Dedifferentiated, RC & pleomorphic liposarcoma (n=119) High grade leiomyosarcoma (n=32) Solitary fibrous tumor (n=21) MPNST Local Recurrence Distant Recurrence

  19. Histology & RP sarcoma Natural History by Histologic Type/Subtype Disease-Specific Death (n=184) Well-diff & myxoid liposarcoma (n=227) Dedifferentiated, RC & pleomorphic liposarcoma (n=119) High grade leiomyosarcoma (n=32) Solitary fibrous tumor (n=21) MPNST Local Recurrence Distant Recurrence

  20. Histology & RP sarcoma Summary • Liposarcoma, leiomyosarcoma and SFT are associated with late recurrence and death from disease (as long as 15 years from initial diagnosis) • Dedifferentiated liposarcoma and high grade leiomyosarcoma have similar DSS, but distinct patterns of local and distant recurrence. • SFT is associated with low local recurrence (<10%) but high distant recurrence (40% at 5 years)

  21. Histology & RP sarcoma Conclusions • Histologic subtypes of RPS have distinct differences in the incidence and timing of local and distant recurrence, as well as disease specific death • Multivariate models and nomograms estimating risk of DSD, LR and DR • Inform patients and physicians of an individual’s risk • May prove useful for selection of patients for clinical trials of: • Neo-adjuvant radiation • Neo-adjuvant/adjuvant chemotherapy • Molecular targeted therapy

  22. Acknowledgements MSKCC Sarcoma Database • July 1982-present • n=10,150 patients • 10,000 patients reached on May 21st, 2013 Funding: • NIH SPORE in Soft Tissue Sarcoma P50 CA 140146-01 • NIH Soft Tissue Sarcoma program project grant P01 CA 047179 • The David and Monica GorinSarcoma Fund

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