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-Xuan Qin, Narasimhan Agaram, Nicole Moraco, Aimée Crago, Samuel Singer Memorial Sloan-Kettering Cancer Center Sarcoma Disease Management Program
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The Importance of Histologic Subtype for Outcome Prediction in Primary Retroperitoneal Sarcoma: A Single-Institution Study of 675 Patients Marcus Tan,
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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-Xuan Qin, Narasimhan Agaram, Nicole Moraco,
Aimée Crago, Samuel Singer
Memorial Sloan-Kettering Cancer CenterSarcoma Disease Management Program
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
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
Histology & RP sarcoma
Methods
• Retrospective review of prospectively-maintained sarcoma database from July 1982 to July 2010
Solitary fibrous tumor 6.3 2.6-15.3 <0.001 Malignant peripheral nerve sheath tumor 2.4 0.6-9.2 0.21 Translocation-associated sarcoma 6.5 2.7-16.0 <0.001Number of Organs Resected 1 or 2 vs. 0 1.4 0.9-2.0 0.1 ≥3 vs. 0 2.4 1.4-4.4 0.002Size 10-20cm vs <10cm 1.8 1.1-2.8 0.01 ≥20cm vs <10cm 1.2 0.7-2.0 0.51
Histology & RP sarcoma
Distant Recurrence Nomogram
DD LPS
Low grade LMS
High grade LMSWD LPS
MPNST
SFT
Other
Concordance index 0.72
Histology & RP sarcoma
Natural History by Histologic Type/Subtype
0 5 10 15 20
0.0
0.4
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Years from Initial Operation
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0 5 10 15 20
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Years from Initial Operation
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0 5 10 15 20
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Years from Initial Operation
Pro
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Histology & RP sarcoma
Well-diff & myxoid liposarcoma
Dedifferentiated, RC & pleomorphic liposarcoma
High grade leiomyosarcoma
Solitary fibrous tumor
MPNST (n=21)
(n=32)
(n=184)
(n=227)(n=119)
Disease-Specific Death
Local Recurrence Distant Recurrence
0 5 10 15 20
0.0
0.4
0.8
Years from Initial Operation
Pro
ba
bili
ty o
f d
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0 5 10 15 20
0.0
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0.8
Years from Initial Operation
Pro
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ty o
f lo
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0 5 10 15 20
0.0
0.4
0.8
Years from Initial Operation
Pro
ba
bili
ty o
f d
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recu
rre
nce
Natural History by Histologic Type/SubtypeHistology & RP sarcoma
Well-diff & myxoid liposarcoma
Dedifferentiated, RC & pleomorphic liposarcoma
High grade leiomyosarcoma
Solitary fibrous tumor
MPNST (n=21)
(n=32)
(n=184)
(n=227)(n=119)
Disease-Specific Death
Local Recurrence Distant Recurrence
0 5 10 15 20
0.0
0.4
0.8
Years from Initial Operation
Pro
ba
bili
ty o
f d
ea
th d
ue
to
dis
ea
se
0 5 10 15 20
0.0
0.4
0.8
Years from Initial Operation
Pro
ba
bili
ty o
f lo
cal r
ecu
rre
nce
0 5 10 15 20
0.0
0.4
0.8
Years from Initial Operation
Pro
ba
bili
ty o
f d
ista
nt
recu
rre
nce
Natural History by Histologic Type/SubtypeHistology & RP sarcoma
Well-diff & myxoid liposarcoma
Dedifferentiated, RC & pleomorphic liposarcoma
High grade leiomyosarcoma
Solitary fibrous tumor
MPNST (n=21)
(n=32)
(n=184)
(n=227)(n=119)
Disease-Specific Death
Local Recurrence Distant Recurrence
0 5 10 15 20
0.0
0.4
0.8
Years from Initial Operation
Pro
ba
bili
ty o
f d
ea
th d
ue
to
dis
ea
se
0 5 10 15 20
0.0
0.4
0.8
Years from Initial Operation
Pro
ba
bili
ty o
f lo
cal r
ecu
rre
nce
0 5 10 15 20
0.0
0.4
0.8
Years from Initial Operation
Pro
ba
bili
ty o
f d
ista
nt
recu
rre
nce
Natural History by Histologic Type/SubtypeHistology & RP sarcoma
Well-diff & myxoid liposarcoma
Dedifferentiated, RC & pleomorphic liposarcoma
High grade leiomyosarcoma
Solitary fibrous tumor
MPNST (n=21)
(n=32)
(n=184)
(n=227)(n=119)
Disease-Specific Death
Local Recurrence Distant Recurrence
0 5 10 15 20
0.0
0.4
0.8
Years from Initial Operation
Pro
ba
bili
ty o
f d
ea
th d
ue
to
dis
ea
se
0 5 10 15 20
0.0
0.4
0.8
Years from Initial Operation
Pro
ba
bili
ty o
f lo
cal r
ecu
rre
nce
0 5 10 15 20
0.0
0.4
0.8
Years from Initial Operation
Pro
ba
bili
ty o
f d
ista
nt
recu
rre
nce
Natural History by Histologic Type/SubtypeHistology & RP sarcoma
Well-diff & myxoid liposarcoma
Dedifferentiated, RC & pleomorphic liposarcoma
High grade leiomyosarcoma
Solitary fibrous tumor
MPNST (n=21)
(n=32)
(n=184)
(n=227)(n=119)
Disease-Specific Death
Local Recurrence Distant Recurrence
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)
Histologic type / subtype
Completeness of resection
Tumor size
Number of organs resected
Age
DSD (R2 vs R0/R1) LR (R1 vs R0) DR
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
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 Gorin Sarcoma Fund