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9 Local recurrence-free survival (RFS), overall recurrence-free survival, and disease-­ specific survival (DSS) by size category, ≤5, 5–10, 10–15, and >15 cm. 001. 001. 91 comparing >10–15 and >15 cm groups. With permission from: Maki RG, et al. Ann Surg Oncol. 2013 20(11):3377–3383 26 2 Natural History: Importance of Size, Site, Histopathology Fig. 10 Disease-specific survival, primary extremity high grade, by size and depth. MSKCC 7/1/1982– 5/31/2013 n = 1808 The AJCC staging system, 8th edition, continues to incorporate the FNCLCC three-­ tier grading system, though the 8th edition of the AJCC system for extremity and trunk tumors still has a dichotomy in that both grade 2 and 3 tumors are considered higher-risk tumors.

Any difference between categories a Relative risk to other categories of the same factor analysis and were associated with a higher risk of local recurrence. 14). Local recurrence for all is approximately 25 %. 17). 6). As with all of these issues, many of these factors are not arbitrary, but interdependent and continuous. 18) shows an increasing risk of disease-specific death. 6 Prognostic factors in extremity soft tissue sarcoma—summary of significant adverse prognostic factors Local recurrence LR at presentation Positive margins MPNST Age >50 Distant recurrence High grade Size >5 cm Size >10 cm Deep location LR at presentation Post-metastasis survival Size >10 cm Disease-specific survival High grade Size >10 cm Deep location Positive margins LR at presentation Lower extremity site MPNST Leiomyosarcoma MSKCC 1982–1994 n = 1041 MPNST malignant peripheral nerve sheath tumor Adapted from: Pisters P, Leung D, Woodruff J, Shi W, Brennan MF.

We have shown that grade, depth, and size are independent predictors of outcome, and most systems base the risk of developing distant metastases giving each factor equal weight. 3 Staging Percent Survived (Log Scale) Fig. 3 1977 AJCC staging system. From: Russell WO, et al. Cancer 40:1562–1570, 1977 21 100 80 60 Fig. 5 Distant disease-­ free survival, primary extremity ≤5 cm, by grade. MSKCC 7/1/1982– 5/31/2013 n = 1039 Stage II (86 Case) 40 Stage III (329 Case) 20 10 8 6 Stage IV (110 Case) 4 2 1 0 Fig.

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