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CTOS 12th Annual Meeting Venice, 2-4 November 2006 Myxoid/RC Liposarcoma: Prognostic Factors And Survival. Marco Fiore marco.fiore@istitutotumori.mi.it. Soft Tissue Sarcoma INT 1980-2005. 23%. N° pz. operated3026 Liposarcoma 685 MFH/Pleomorphic sarcoma 389 - PowerPoint PPT Presentation
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CTOS 12th Annual MeetingVenice, 2-4 November 2006
Myxoid/RC Liposarcoma: Prognostic Factors And Survival
Marco Fioremarco.fiore@istitutotumori.mi.it
Soft Tissue SarcomaINT 1980-2005
N° pz. operated 3026
Liposarcoma 685MFH/Pleomorphic sarcoma 389Leiomiosarcoma 372DFSP 260Aggressive Fibromatosis 240Synovial Sarcoma 224MPNST 222Sarcoma NOS 143 Others 491
23%
WD Liposarcoma 233
Dediff Liposarcoma 123
Myxoid Liposarcoma 202
Round cell Liposarcoma 66
Pleomorphic Liposarcoma 61
685 Liposarcoma INT 1980-2005
48%
FUS/TLS-CHOP t(12;16)(q13;p11) (95%)
FUS/TLS
EWS
CHOP/DDIT3
EWS-CHOP t(12;22)(q13;q12) (5%)
myxoid
round cell
pleomorphic
329 Myxoid/RC Liposarcoma INT 1980-2005
2 1%
15 5%
39 12%
273 83%
Head & Neck
Trunk
Retr./Intrapelvic
Extremities
75%
Median Age 49 yrsMale : Female 1 : 1Median Size 8.5 cmDeep Site 84%Grade (FNCLCC)
I 54%II 23%III 22%
329 Myxoid/RC Liposarcoma INT 1980-2005
Primary Recurrent
Total 214 65% 115 35%
MLPS 132 62% 70 61%
RCLPS 44 21% 22 19%
PLPS 38 18% 23 20%
RTNot Done 130 61% 71 62%
Done 84 39% 44 38%
CTNot Done 175 82% 93 81%
Done 39 18% 22 19%
Primary Recurrent
Total 214 65% 115 35%
Site**Extremities 190 89% 83 72%
Intrabd/RP 16 7% 23 20%
Surgical ProcedureLimb Sparing 177 83% 75 65%
Amputation 5 2% 6 5%
NA (trunk, H&N, RP) 32 15% 34 30%
Surgical Margins**Negative 186 87% 75 65%
Positive 28 13% 40 35%
Prognosis depends on…
•the tumor
Hazard Ratio 95% C. I.
Presentation: Recurrence ( Primary ) 2.781** 1.659 4.661
Depth: Deep ( Superficial ) 1.632** 0.556 4.794
Size : >5 cm ( 5 cm ) 2.161** 0.911 5.124
Site: Extremity ( Other ) 0.580** 0.317 1.062
Grade: II ( I ) 2.356** 1.107 5.013
Grade: III ( I ) 1.853** 0.701 4.899
Histology: PLPS (MLPS ) 0.908** 0.369 2.232
Multivariate Analysis - DSS
0
0.1
0.2
0.3
0.4
0.5
0.6
0.7
0.8
0.9
1 STS high gradeDFS
Suit HD. J Clin Oncol 1988; 6: 854
<2,5 2,5-5 5-10 10-15 15-20 >20 cm
Size…
Site…
0
0.2
0.4
0.6
0.8
1
0 1 2 3 4 5 6 7 aa
grade 1
grade 2
grade 3
OS
Grading…
Round cell component…
Antonescu CR, Clin Cancer Res 2001;7:3977
RC >5%
Primary: Disease-Specific Survival
Years
0 1 2 3 4 5 6 7 8 9 10
Pro
babi
lity
0.0
0.1
0.2
0.3
0.4
0.5
0.6
0.7
0.8
0.9
1.0
MLPSRCLPSPLPS
0.2217P
214 Primary Myxoid/RC LPS - DSS INT 1980-2005
5-yrs 10-yrs
Overall 90% 87%
MLPS 93% 90%
RCLPS 87% 81%
PLPS 82% 82%
Primary: Crude Cumulative Incidence of Local-Regional Relapse
Years
0 1 2 3 4 5 6 7 8 9 10
Pro
bab
ility
0.0
0.1
0.2
0.3
0.4
0.5
0.6MLPSRCLPSPLPS
0.8907P
214 Primary Myxoid/RC LPS - LR INT 1980-2005
5-yrs 10-yrs
Overall 13% 18%
MLPS 12% 18%
RCLPS 10% 15%
PLPS 19% 19%
Primary: Crude Cumulative Incidence of Distant Metastasis
Years
0 1 2 3 4 5 6 7 8 9 10
Pro
ba
bilit
y
0.0
0.1
0.2
0.3
0.4
0.5
0.6MLPSRCLPSPLPS
0.0018P
214 Primary Myxoid/RC LPS - DM INT 1980-2005
5-yrs 10-yrs
Overall 10% 11%
MLPS 4% 5%
RCLPS 23% 23%
PLPS 20% 20%
p=.0018
Pattern of Distant Mets
41% extra-pulmonary mets
26% lung mets
33% both
• 74% of metastatic patients had at least one extra-pulmonary mets
Extra-pulmonary metastatic pattern according to histological subtype.
MLPS RCLPS PLPS Overall
Intrabdominal 6 4/2 5 3/2 - - 11 7/4
Bone/Spine 6 2/4 3 2/1 - - 9 4/5
Paraspinal 3 0/3 5 2/3 - - 8 2/6
Extremities 4 2/2 3 2/1 1 1/0 8 5/3
Soft tissues 1 0/1 4 3/1 - - 5 3/2
Thoracic wall 3 2/1 2 0/2 - - 5 2/3
Retroperitoneum 3 1/2 1 0/1 - - 4 1/3
Liver 1 1/0 1 0/1 2 2/0 4 3/1
Heart/Pericardium 2 1/1 1 0/1 1 1/0 4 2/2
Mediastinum 2 1/1 2 2/0 - - 4 3/1
Brain - - 1 0/1 1 1/0 2 1/1
Regional nodes 1 0/1 1 0/1 - - 2 0/2
Abdominal wall 1 0/1 - - - - 1 0/1
Overall 33 14/19 29 14/15 5 5/0 67 33/34
Multiple extra-pulmonary sites 8 4/4 6 5/1 - - 14 9/5
* Number of localization at single sites (primary/recurrent cases) in 43 patients with extrapulmonary metastases.
49% 43%
33%
Local Relapse
Distant Mets
MLPS 5 – 9636
8 – 7215
RCLPS 8 – 6612
6 – 5420
PLPS 4 – 3012
6 – 4818
Time to Event (mos)
Local Relapse
Point Estimate 95% CI
MLPS >266 143 - >266
RCLPS 103 46 - >158
PLPS >97 90 - >277
Post-Event Median Survival (mos)
Lung Mets Extra-Lung Mets
Point Estimate 95% CI Point
Estimate 95% CI
MLPS 23 7 - 39 14 4 - >52
RCLPS >12 . - . >19 5 - >19
PLPS 13 6 - 98 >42 >42 - >42
Post-Event Median Survival (mos)
•the surgeon
Intralesional 100%
Marginal 60-80%
Wide 10-30%
Radical < 5%
LR
Enzinger and Weiss’s SOFT TISSUE TUMORS - 2001
Margins status: +/- 2.161** 1.2333.78
7 0.0071
Hazard Ratio 95% C. I. P
Multivariate Analysis - DSS
Local recurrence has an independent effect on
prognosis
Recurrent: Disease-Specific Survival
Years
0 1 2 3 4 5 6 7 8 9 10
Pro
babi
lity
0.0
0.1
0.2
0.3
0.4
0.5
0.6
0.7
0.8
0.9
1.0
MLPSRCLPSPLPS
0.1512P
115 Recurrent Myxoid/RC LPS - DSS INT 1980-2005
5-yrs 10-yrs
Overall 72% 56%
MLPS 79% 62%
RCLPS 58% 43%
PLPS 64% 53%
Recurrent: Crude Cumulative Incidence of Local-Regional Relapse
Years
0 1 2 3 4 5 6 7 8 9 10
Pro
babi
lity
0.0
0.1
0.2
0.3
0.4
0.5
0.6
MLPSRCLPSPLPS
0.2837P
115 Recurrent Myxoid/RC LPS - LR INT 1980-2005
5-yrs 10-yrs
Overall 37% 39%
MLPS 35% 37%
RCLPS 31% 31%
PLPS 48% 52%
Recurrent: Crude Cumulative Incidence of Distant Metastasis
Years
0 1 2 3 4 5 6 7 8 9 10
Pro
ba
bilit
y
0.0
0.1
0.2
0.3
0.4
0.5
0.6MLPSRCLPSPLPS
0.0164P
115 Recurrent Myxoid/RC LPS - DM INT 1980-2005
5-yrs 10-yrs
Overall 22% 23%
MLPS 19% 19%
RCLPS 44% 44%
PLPS 13% 17%
… in conclusion
Prognosis of myxoid/round cell liposarcoma is relatively good, if compared with other STS
Cut-off of 5% of RC component single out a group tumor with 81% 10-yrs DSS, which is actually in the range of intermediate grade STS
As expected, phase at presentation, tumor size and grade, and surgical margins are independent prognostic factors for survival
Pattern of metastases can affect survival
… marco.fiore@istitutotumori.mi.it
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