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Myxofibrosarcoma: a retrospective analysis of 158 patients

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 Sanfilippo roberta.sanfilippo@istitutotumori.mi.it. Myxofibrosarcoma. INT, Milano May 1994 - February 2009.

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Myxofibrosarcoma: a retrospective analysis of 158 patients

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  1. 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 Sanfilippo roberta.sanfilippo@istitutotumori.mi.it

  2. Myxofibrosarcoma

  3. INT, Milano May 1994 - February 2009

  4. 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%

  5. Patients’ characteristics Chemotherapy Done 14% Not Done 86% Radiotherapy Done 51% Not Done 49% Primary tumor Recurrent tumor

  6. Tumor sites Head&neck 1% Trunk 14% Upper extremities 24% Lower extremities 61%

  7. Surgery Surgical Procedure • Conservative 157/158 • Amputation 1/158 (1/132) extremities Surgical margins • Negative 82% • Positive 18%

  8. 1.0 0.8 0.6 0.4 0.2 0.0 0 12 24 36 48 60 OS Survival probability OS@5yrs=77% Time (months) Median follow-up (from surgery): 53 months

  9. Multivariate analysis for OS HRP • 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

  10. 0.20 0.18 0.16 0.14 0.12 0.10 0.08 0.06 0.04 0.02 0.00 0 12 24 36 48 60 LR and M Crude cumulative incidence Distant metastasis Local relapse Probability LR@5yrs=18% DM@5yrs=15% Time (months) Median follow-up (from surgery): 53 months

  11. Univariate analysis for LR HRP • 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

  12. Multivariate analysis for LR HRP • 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

  13. Multivariate analysis for M HRP • 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 **

  14. 1.0 0.8 0.6 0.4 0.2 0.0 0 12 24 36 48 60 OS Myxofibrosarcoma G3 Survival probability % OS@5yrs=64.5% Time (months)

  15. 0.30 0.28 0.26 0.24 0.22 0.20 0.18 0.16 0.14 0.12 0.10 0.08 0.06 0.04 0.02 0.00 0 12 24 36 48 60 LR and M in G3 Myxofibrosarcoma Distant metastasis Probability Local relapse LR@5yrs=11% DM@5yrs=27% Time (months)

  16. Patterns of metastasis 20%

  17. “Infiltrative margins”

  18. Margins and size Median size • Positive margins (I.Q. range) 9.5 (5-16.5) cm • Negative margins (I.Q. range) 5 (3-7) cm

  19. 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

  20. roberta.sanfilippo@istitutotumori.mi.it alessadro.gronchi@istitutotumori.mi.it paolo.casali@istitutotumori.mi.it

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