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Emily Z Keung, 1 Yang Feng, 2 Jason L Hornick, 3 Judith Manola, 2

Clinicopathologic Characteristics and Predictors of Outcomes in Patients with Primary Retroperitoneal Dedifferentiated Liposarcoma Undergoing Surgery. Emily Z Keung, 1 Yang Feng, 2 Jason L Hornick, 3 Judith Manola, 2 Monica M Bertagnolli, 1 Elizabeth H Baldini, 4 Chandrajit P Raut 1

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Emily Z Keung, 1 Yang Feng, 2 Jason L Hornick, 3 Judith Manola, 2

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  1. Clinicopathologic Characteristics and Predictors of Outcomes in Patients with Primary Retroperitoneal Dedifferentiated Liposarcoma Undergoing Surgery Emily Z Keung,1 Yang Feng,2 Jason L Hornick,3 Judith Manola,2 Monica M Bertagnolli,1 Elizabeth H Baldini,4 Chandrajit P Raut1 Departments of 1Surgery, 2Biostatistics, 3Pathology, and 4Radiation Oncology Brigham and Women’s Hospital, Dana-Farber Cancer Institute, and Harvard Medical School Boston, Massachusetts, USA

  2. Retroperitoneal Dedifferentiated Liposarcoma Disclosures • The authors have no disclosures to report

  3. Retroperitoneal Dedifferentiated Liposarcoma Dedifferentiated Liposarcoma of the Retroperitoneum • Liposarcoma (LPS) is the most common histology among retroperitoneal sarcomas • Dedifferentiated liposarcoma (DDLPS) is 1 of 4 LPS subtypes • DDLPS of the retroperitoneum (RP DDLPS) • Aggressive tumors with 15-20% metastatic rate • Associated with poor patient prognosis • 90% diagnosed at first presentation (primary DDLPS) • 10% diagnosed in recurrent WDLPS (secondary DDLPS) Dei Tos AP, 2000, Ann Diagn Pathol Ghadimi MP, et al, 2011, Ann Surg Oncol Murphey, et al, 2005, Radiographics Weaver J, et al, 2008, Modern Pathology

  4. Retroperitoneal Dedifferentiated Liposarcoma RP DDLPS: Current Knowledge Deficit • To date, studies examining RPS generally include multiple histologies and those examining DDLPS include multiple disease locations • Several studies have compared WDLPS and DDLPS • DDLPS are associated with • Higher local recurrence • Higher distant recurrence • Decreased survival • No study has examined RP DDLPS alone Lahat G, et al, 2008, Ann Surg Onc Singer S, et al, 2003, Ann Surg

  5. Retroperitoneal Dedifferentiated Liposarcoma Study Objective and Methods • Objective: To identify factors predictive of outcomes within a cohort exclusively of primary RP DDLPS

  6. Retroperitoneal Dedifferentiated Liposarcoma Study Objective and Methods • Objective: To identify factors predictive of outcomes within a cohort exclusively of primary RP DDLPS • A retrospective review of all patients with primary RP DDLPS treated at our institution between 1998 and 2008 was performed (n=119)

  7. Retroperitoneal Dedifferentiated Liposarcoma Study Objective and Methods • Objective: To identify factors predictive of outcomes within a cohort exclusively of primary RP DDLPS • A retrospective review of all patients with primary RP DDLPS treated at our institution between 1998 and 2008 was performed (n=119) • Endpoints of interest: • Progression-free survival (PFS) • Local recurrence-free survival (LRFS) • Distant recurrence-free survival (DRFS) • Overall survival (OS) • Survival was calculated from date of diagnosis by the Kaplan-Meier method

  8. Retroperitoneal Dedifferentiated Liposarcoma Study Objective and Methods • Objective: To identify factors predictive of outcomes within a cohort exclusively of primary RP DDLPS • A retrospective review of all patients with primary RP DDLPS treated at our institution between 1998 and 2008 was performed (n=119) • Endpoints of interest: • Progression-free survival (PFS) • Local recurrence-free survival (LRFS) • Distant recurrence-free survival (DRFS) • Overall survival (OS) • Survival was calculated from date of diagnosis by the Kaplan-Meier method • Multivariable Cox regression analyses were performed to identify significant prognostic factors

  9. Retroperitoneal Dedifferentiated Liposarcoma Patient Characteristics (n=119)

  10. Retroperitoneal Dedifferentiated Liposarcoma Treatment Regimens • Site of initial surgery • Referring institution - 82 patients (69%) • Of these, 19 patients (23%) underwent surgery by urologists or gynecologists • BWH/DFCI – 37 patients (31%)

  11. Retroperitoneal Dedifferentiated Liposarcoma Surgical Outcomes

  12. Retroperitoneal Dedifferentiated Liposarcoma Survival Outcomes

  13. Retroperitoneal Dedifferentiated Liposarcoma High Local Recurrence Rate • 100/119 patients (84%) had disease recurrence or progression • 92/100 (92%) were local recurrences

  14. Retroperitoneal Dedifferentiated Liposarcoma High Local Recurrence Rate • 100/119 patients (84%) had disease recurrence or progression • 92/100 (92%) were local recurrences • Recurrence/progression by extent of resection • R0/R1 resection – 82% had disease recurrence • R2 resection – 77% had disease progression

  15. Retroperitoneal Dedifferentiated Liposarcoma High Local Recurrence Rate • 100/119 patients (84%) had disease recurrence or progression • 92/100 (92%) were local recurrences • Recurrence/progression by extent of resection • R0/R1 resection – 82% had disease recurrence • R2 resection – 77% had disease progression • Distant metastasis uncommon – 22/119 patients (19%)

  16. Retroperitoneal Dedifferentiated Liposarcoma Predictors of Outcomes: Variables Examined • Age • Gender • Location of initial surgery (BWH v. OSH) • DDLPS size • Multifocality • FNCLCC grade • Extent of resection (R0/R1 v. R2) • Tumor integrity (fragmented v. intact tumor specimen) • Chemotherapy at initial presentation • Radiation therapy at initial presentation • Number of organs resected

  17. Retroperitoneal Dedifferentiated Liposarcoma Predictors of PFS

  18. Retroperitoneal Dedifferentiated Liposarcoma PFS: Tumor Integrity at Resection Fragmented tumor specimen Intact tumor specimen

  19. Retroperitoneal Dedifferentiated Liposarcoma Predictors of LRFS

  20. Retroperitoneal Dedifferentiated Liposarcoma LRFS: Location of Initial Surgery and Multifocality Initial surgery at OSH Initial surgery at BWH/DFCI Multifocal disease Single tumor

  21. Retroperitoneal Dedifferentiated Liposarcoma Predictors of DRFS

  22. Retroperitoneal Dedifferentiated Liposarcoma Predictors of OS

  23. Retroperitoneal Dedifferentiated Liposarcoma OS: Grade, Extent of Resection, and Tumor Integrity High grade Intermediate grade R2 resection R0/R1 resection Fragmented tumor specimen Intact tumor specimen

  24. Retroperitoneal Dedifferentiated Liposarcoma Strengths and Limitations • Limitations • Single institution retrospective study • Modest sample size • Strengths • Largest published cohort of patients with RP DDLPS • To our knowledge, this is the first study to examine primary RP DDLPS specifically • Thus, this study provides important prognostic information for the clinician evaluating a patient with primary RP DDLPS in the clinic

  25. Retroperitoneal Dedifferentiated Liposarcoma Conclusions • Primary RP DDLPS are large tumors (median size 20.5 cm), typically requiring multi-organ resection • Resections are often macroscopically complete (R0/R1, ~80%) but margins are rarely negative (~10%)

  26. Retroperitoneal Dedifferentiated Liposarcoma Conclusions • Primary RP DDLPS are large tumors (median size 20.5 cm), typically requiring multi-organ resection • Resections are often macroscopically complete (R0/R1, ~80%) but margins are rarely negative (~10%) • Rate of recurrence/progression is high (84%) • Local recurrence is common (92%) and often multifocal • Distant recurrence is less common (19%)

  27. Retroperitoneal Dedifferentiated Liposarcoma Conclusions – Multivariable Analysis • Initial surgery at BWH/DFCI was associated with better LRFS • Multifocal disease was associated with worse LRFS • High FNCLCC grade and R2 resection were associated with worse OS • Tumor fragmentation during resection was associated with worse PFS and OS • Neither chemotherapy nor radiation therapy at initial presentation was associated with PFS, LRFS, DRFS, or OS

  28. Retroperitoneal Dedifferentiated Liposarcoma Surgical Treatment of 119 Patients with RP DDLPS at Initial Presentation

  29. Retroperitoneal Dedifferentiated Liposarcoma Treatment of DDLPS Recurrence

  30. Retroperitoneal Dedifferentiated Liposarcoma FNCLCC Grading System

  31. Retroperitoneal Dedifferentiated Liposarcoma Outcomes of 119 Patients with Primary RP DDLPS

  32. Retroperitoneal Dedifferentiated Liposarcoma Predictors of LRFS, DRFS, PFS, and OS Among Patients Undergoing R0/R1 Resection: Multivariable Cox Regression Analyses

  33. Retroperitoneal Dedifferentiated Liposarcoma Retroperitoneal Sarcomas (RPS) • Retroperitoneal sarcomas constitute ~10-15% of all soft tissue sarcoma with incidence of 2-5 per million • Surgical resection is the only curative treatment • Local recurrence is common (5-year local recurrence ~70%) and accounts for nearly 90% of disease-related mortality (5-year overall survival ~50%) • Liposarcomas account for the majority of RPS with multiple histologic subtypes: • Well-differentiated • Dedifferentiated • Myxoid/round cell • Pleomorphic Anaya DA, et al. Ann Surg Oncol. 2009;16:667-675. Crago AM, Singer S. Curr Opin Oncol. 2011;23(4):373-378. Mussi C, et al. Cancer. 2008;113:1657-1665.

  34. Retroperitoneal Dedifferentiated Liposarcoma Retroperitoneal Dedifferentiated Liposarcoma (RP DDLPS): The Current Knowledge Deficit • To date, studies examining RPS and DDLPS include either multiple histologies and/or disease locations • A few studies have included subsets of patients with RP DDLPS • No study has been performed examining RP DDLPS alone

  35. Retroperitoneal Dedifferentiated Liposarcoma Conclusions • Nearly 1/4 of patients presenting to referring institutions underwent surgery by urologists or gynecologists • It is important that these surgeons consider RP DDLPS when evaluating patients with an RP mass and be familiar with the management of RP DDLPS

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