Diagnosis and management of retroperitoneal sarcoma

Transcription

1 SON Update 2017 Diagnosis and management of retroperitoneal sarcoma Andrea J MacNeill, MD MSc FRCSC Surgical Oncologist, BC Cancer Agency Vancouver

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3 Histologic Subtypes of STS 3 RP Subtypes (n=684) Extremity Subtypes (n=3,039) Desmoid (2%) Fibrosarcoma (1%) Leiomyosarcoma (21%) Liposarcoma (59%) MPNST (3%) MFH (3%) Sarcoma-NOS (2%) SFT/Hemangiopericytoma (2%) Undifferentiated (1%) Other (5%) DFSP (3%) Desmoid (5%) Leiomyosarcoma (8%) Liposarcoma (23%) MPNST (3%) MFH (28%) Synovial (11%) Other (19%) MSKCC STS Database

4 Outcomes in STS 4 Overall Survival after Resection by Primary Site of STS (Princess Margaret Hospital) Catton, O Sullivan et al., Int J Rad Oncol Biol Phys 1994; 29:1005.

5 Local failure kills 5 Local failure leading cause of disease-specific mortality Anatomic constraints often preclude R0 resection Complete (R0/1) vs Incomplete (R2)

6 6 Evolution of Surgical Approach to RPS

7 Extended resection leads to improved 7 local control fig 1. Crude cumulative incidence of local recurrence by period of surgical resection at a single institution.

8 Extended resection is associated with improved 8 overall survival 66% 48% Gronchi et al., Ann Oncol 2012.

9 Extended resection 9 operative approach Principle liberal resection of all involved organs Right side RPS Left side RPS Colon Kidney Psoas fascia +/- Duodenum +/- Liver capsule +/- IVC/iliac vessels Colon Kidney Psoas fascia Distal pancreas + spleen +/- Aorta/iliac vessels +/- Diaphragm

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21 How to work up a 21 retroperitoneal mass Labs Imaging B-HCG AFP LDH Metanephrines/catecholamines if appropriate CT abdomen preferable to MRI CT chest for staging Differential renal scan if nephrectomy anticipated

22 22 Why Biopsy? Establish diagnosis: eliminate nonoperative pathology

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26 26 Why Biopsy? Establish diagnosis: eliminate nonoperative pathology Identify histologic subtype: tailor treatment strategy

27 27 How do histologic subtype and management strategy AFFECT RECURRENCE?

28 Local Recurrence 28 5y LR 24% 10y LR 33%

29 Distant Metastasis 29 5y DM 21% 10y DM 21.6%

30 30 Institutional Management Strategies: WDLPS

31 31 Institutional Management Strategies: LMS

32 32 Why Biopsy? Establish diagnosis: eliminate nonoperative pathology Identify histologic subtype: tailor treatment strategy Consider neoadjuvant therapy: radiate first

33 33 Radiation Therapy in RPS Goal: Establish Increase R0 resection rate diagnosis: Decrease local recurrence rate eliminate Identify histologic subtype: Level 1 evidence of improved local control in nonoperative extremity STS, no survival benefit pathology tailor treatment strategy EORTC study STRASS - A phase III randomized study of preoperative radiotherapy plus surgery versus surgery alone for patients with Retroperitoneal sarcoma (RPS)

34 34 Pre-op RT More accurate targeting of tumour volume Minimal toxicity Improved delivery to welloxygenated tissues Increased likelihood of negative margin Reduced chance of intraoperative contamination/tumour rupture

35 35 Pre-op RT Post-op RT More accurate targeting of tumour NOT POSSIBLE! volume Minimal toxicity Improved delivery to welloxygenated tissues Increased likelihood of negative margin Reduced chance of intraoperative contamination/tumour rupture

36 Pre-op 36 Post-op Post-op

37 37 How (not) to Biopsy? Establish At diagnosis: sarcoma referral centre eliminate nonoperative pathology Via RP approach Targeting dedifferentiated areas With expert pathology review

38 38 How (not) to Biopsy? Establish At diagnosis: sarcoma referral centre eliminate nonoperative Via RP approach No pathology role for transperitoneal/ laparoscopic/open biopsy Targeting dedifferentiated areas With expert pathology review

39 39 Principles and Pitfalls Establish diagnosis: eliminate Identify histologic Best outcomes achieved at high-volume centres subtype: nonoperative pathology tailor treatment strategy

40 Overall Survival TARPSWG centres 40 5y OS 67% 10y OS 46%

41 41 Principles and Pitfalls Establish diagnosis: eliminate Identify Best outcomes achieved at high-volume centres All RPS require MDC review histologic subtype: nonoperative pathology tailor treatment strategy

42 42 Principles and Pitfalls Establish diagnosis: eliminate Identify Best outcomes achieved at high-volume centres All RPS require MDC review histologic subtype: nonoperative pathology tailor treatment strategy Large mass emergency

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44 44 Principles and Pitfalls Establish diagnosis: eliminate Identify Best outcomes achieved at high-volume centres All RPS require MDC review histologic subtype: nonoperative pathology tailor treatment strategy Large mass emergency Incidental finding at laparotomy/inguinal hernia repair do not biopsy, avoid mesh

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46 46 Take home messages Establish RPS is a family of diseases diagnosis: Identify histologic eliminate subtype: Management is multidisciplinary and must be nonoperative tailored to histology tailor treatment pathology strategy Preoperative tissue diagnosis is imperative Outcomes have improved with extended resection, referral to high-volume centres