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Diagnosis and management of
retroperitoneal sarcoma
SON Update 2017
Andrea J MacNeill, MD MSc FRCSC
Surgical Oncologist, BC Cancer Agency
Vancouver
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Histologic Subtypes of STS
MSKCC STS Database
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%)
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Outcomes in STS
Catton, O’Sullivan et al., Int J Rad Oncol Biol Phys 1994; 29:1005.
Overall Survival after Resection by
Primary Site of STS
(Princess Margaret Hospital)
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Local failure kills
• Local failure leading cause of disease-specific mortality
• Anatomic constraints often preclude R0 resection
✓ Complete (R0/1) vs Incomplete (R2)
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Evolution
of Surgical
Approach to
RPS
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Extended resection leads to improved
local control
fig 1. Crude cumulative incidence of local
recurrence by period of surgical resection at
a single institution.
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Extended resection is associated with improved
Gronchi et al., Ann Oncol 2012.
66%
overall survival
48%
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Extended resection
operative approachPrinciple – liberal resection of all “involved” organs
• Colon
• Kidney
• Psoas fascia
• +/- Duodenum
• +/- Liver capsule
• +/- IVC/iliac vessels
Right side RPS
• Colon
• Kidney
• Psoas fascia
• Distal pancreas + spleen
• +/- Aorta/iliac vessels
• +/- Diaphragm
Left side RPS
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How to work up a
retroperitoneal mass
Labs
• CT abdomen preferable to MRI
• CT chest for staging
• Differential renal scan if nephrectomy
anticipated
Imaging
• B-HCG
• AFP
• LDH
• Metanephrines/catecholamines if
appropriate
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Why
Biopsy? Establish
diagnosis:eliminate
nonoperative
pathology
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Why
Biopsy? Establish
diagnosis:eliminate
nonoperative
pathology
Identify
histologic
subtype:tailor treatment
strategy
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How do histologic subtype
and management strategy
AFFECT
RECURRENCE?
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Local Recurrence
5y LR 24%
10y LR 33%
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Distant Metastasis
5y DM 21%
10y DM 21.6%
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Institutional
Management
Strategies:
WDLPS
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Institutional
Management
Strategies:
LMS
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Why
Biopsy?
Consider
neoadjuvant
therapy:radiate first
Establish
diagnosis:eliminate
nonoperative
pathology
Identify
histologic
subtype:tailor treatment
strategy
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Radiation
Therapy
in RPS
Establish
diagnosis:eliminate
nonoperative
pathology
Identify
histologic
subtype:tailor treatment
strategy
EORTC study 62092-22092
STRASS - A phase III randomized study of preoperative
radiotherapy plus surgery versus surgery alone for patients
with Retroperitoneal sarcoma (RPS)
Goal:
• Increase R0 resection rate
• Decrease local recurrence rate
Level 1 evidence of improved local control in
extremity STS, no survival benefit
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• More accurate targeting of tumour
volume
• Minimal toxicity
• Improved delivery to well-
oxygenated tissues
• Increased likelihood of negative
margin
• Reduced chance of intraoperative
contamination/tumour rupture
Pre-op RT
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• More accurate targeting of tumour
volume
• Minimal toxicity
• Improved delivery to well-
oxygenated tissues
• Increased likelihood of negative
margin
• Reduced chance of intraoperative
contamination/tumour rupture
• NOT POSSIBLE!
Pre-op RT Post-op RT
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36Pre-op
Post-op
Post-op
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How (not)
to Biopsy? Establish
diagnosis:eliminate
nonoperative
pathology
Targeting
dedifferentiated
areas
Via RP
approach
At sarcoma
referral centre
With expert
pathology
review
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How (not)
to Biopsy? Establish
diagnosis:eliminate
nonoperative
pathology
Targeting
dedifferentiated
areas
Via RP
approach
At sarcoma
referral centre
With expert
pathology
review
No role for transperitoneal/
laparoscopic/open biopsy
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Principles
and
Pitfalls
Establish
diagnosis:eliminate
nonoperative
pathology
Identify
histologic
subtype:tailor treatment
strategy
• Best outcomes achieved at high-volume centres
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Overall Survival – TARPSWG centres
5y OS 67%
10y OS 46%
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Principles
and
Pitfalls
Establish
diagnosis:eliminate
nonoperative
pathology
Identify
histologic
subtype:tailor treatment
strategy
• Best outcomes achieved at high-volume centres
• All RPS require MDC review
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Principles
and
Pitfalls
Establish
diagnosis:eliminate
nonoperative
pathology
Identify
histologic
subtype:tailor treatment
strategy
• Large mass ≠ emergency
• Best outcomes achieved at high-volume centres
• All RPS require MDC review
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Principles
and
Pitfalls
Establish
diagnosis:eliminate
nonoperative
pathology
Identify
histologic
subtype:tailor treatment
strategy
• Large mass ≠ emergency
• Incidental finding at laparotomy/inguinal
hernia repair – do not biopsy, avoid mesh
• Best outcomes achieved at high-volume centres
• All RPS require MDC review
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Take home
messages Establish
diagnosis:eliminate
nonoperative
pathology
Identify
histologic
subtype:tailor treatment
strategy
• RPS is a family of diseases
• Management is multidisciplinary and must be
tailored to histology
• Preoperative tissue diagnosis is imperative
• Outcomes have improved with extended
resection, referral to high-volume centres