Myxofibrosarcoma:

a retrospective analysis of 158 patients

Sanfilippo R, Grosso F, Pennacchioli E, Barisella M, Fiore M, Morosi C, Collini P, Casali PG, Gronchi A

Roberta Sanfilipporoberta.sanfilippo@istitutotumori.mi.it

Myxofibrosarcoma

INT, Milano May 1994 - February 2009

Patients’ characteristics Median age (I.Q. range) 64 yrs (54-72)

Sex Male 56% Female 44%

Median size(I.Q. range) 5 (3-9) cm

Deep site 56%

Grade (FNCLCC)grade I 22%

grade II 42%

grade III 36%

Patients’ characteristics

Primary tumor

Recurrent tumor

Chemotherapy

Done 14%Not Done 86%

RadiotherapyDone 51% Not Done 49%

Tumor sites

Head&neck 1%

Trunk 14%

Upper extremities 24%

Lower extremities 61%

Surgery

Surgical Procedure

Conservative 157/158

Amputation 1/158 (1/132)

Surgical margins

Negative 82%

Positive 18%

extremities

OS

0 12 24 36 48 60

0.0

0.2

0.4

0.6

0.8

1.0

Time (months)

Su

rviv

al p

rob

abil

ity

OS@5yrs=77%

Median follow-up (from surgery): 53 months

Presentation: recurrent 1.82 (0.79, 4.17) 0.159

vs primary

Depht: deep vs superficial 0.76 (0.30, 1.91) 0.558

Size: 9 vs 3 cm 3.46 (1.13,10.62) 0.005

Grade : III vs II&I 4.76 (2.15, 10.54) <0.0001

Margins: positive vs negative 2.51 (1.08,5.82) 0.032

RT: done vs not done 1.31 (0.58,2.97) 0.522

CT: done vs not done 0.65 (0.22,1.88) 0.422

Multivariate analysis for OS

HR P

LR and M

0 12 24 36 48 60

0.00

0.02

0.04

0.06

0.08

0.10

0.12

0.14

0.16

0.18

0.20

Time (months)

Pro

bab

ilit

yCrude cumulative incidence

Distant metastasis

Local relapse

Median follow-up (from surgery): 53 months

LR@5yrs=18%DM@5yrs=15%

Univariate analysis for LR HR P

Presentation: recurrent 1.03 (0.43, 2.43) 0.95

vs primary

Depht: deep vs superficial 1.50 (0.69,3.28) 0.3081

Size: 9 vs 3 cm 1.23(0.51-2.96) 0.115

Site: extremity vs other 1.56 (0.47, 5.18) 0.4691

Grade : II vs I 0.67 (0.29, 1.55)0.3472

III vs II 0.46 (0.16, 1.35)

Margins: positive vs negative 5.40 (2.46, 11.86) <.0001

RT: done vs not done 1.36 (0.63, 2.91) 0.4321

CT: done vs not done 1.87 (0.64, 5.49) 0.2555

Presentation: recurrent 0.61 (0.23, 1.60) 0.314

vs primary

Depht: deep vs superficial 1.08 (0.41, 2.82) 0.875

Size: 9 vs 3 cm 0.87 (0.30,2.56) 0.859

Margins: positive vs negative 5.63 (2.06,15.38) <0.001**

Grade : III vs II&I 0.80 (0.29, 2.21) 0.662

RT: done vs not done 1.07 (0.47,2.44) 0.863

Multivariate analysis for LR

HR P

Presentation: recurrent 1.74 (0.66, 4.57) 0.264 vs primary

Depht: deep vs superficial 1.55 (0.47, 5.09) 0.475

Size: 9 vs 3 cm 1.18(0.32-4.32) 0.728

Grade : III vs I/II 5.35 (1.93, 14.84) 0.001

Margins: positive vs negative 2.86 (0.90,9.06) 0.074

CT: done vs not done 0.84 (0.27,2.62) 0.760

Multivariate analysis for M

HR P

**

OS Myxofibrosarcoma G3

0 12 24 36 48 60

0.0

0.2

0.4

0.

60

.81

.0

Su

rviv

al p

rob

abil

ity

%

Time (months)

OS@5yrs=64.5%

0 12 24 36 48 60

0.00

0.02

0.04

0.06

0.08

0.10

0.12

0.14

0.16

0.18

0.20

0.22

0.24

0.26

0.28

0.30

Pro

bab

ilit

y

LR and M in G3 Myxofibrosarcoma

Distant metastasis

Local relapse

Time (months)

LR@5yrs=11%DM@5yrs=27%

Patterns of metastasis

20%

“Infiltrative margins”

Positive margins (I.Q. range) 9.5 (5-16.5) cm

Negative margins (I.Q. range)5 (3-7) cm

Median size

Margins and size

Conclusions Myxofibrosarcomas have a good prognosis, and

even high-grade ones have a comparatively low risk of metastases

By contrast, the local relapse rate is definetely higher, likely due to the typically higher infiltrative pattern of growth

However, the local recurrence rate does not seem to influence the outcome

Though in the presence of an obvious selection bias in this series, the role of radiation therapy is left to be defined

roberta.sanfilippo@istitutotumori.mi.italessadro.gronchi@istitutotumori.mi.it

paolo.casali@istitutotumori.mi.it