Table 1:
Author | Year | N | Median age (y, range) | Gender (male / female) | Median tumor diameter (cm, range) | Location | Primary or recurrent | Histologic subtype | Inclusion criteria | Exclusion criteria |
---|---|---|---|---|---|---|---|---|---|---|
Ugurel et al | 2014 | 14* | 51.3 (27 – 72) | 3 / 11 | 4.3 (1.7 – 18.3) | Trunk, extremities | 11/14 primary | 2/14 FS | Locally advanced DFSP but surgically manageable |
Metastasis |
Wang et al | 2014 | 22 | 43 (21 – 69) | 9 / 13 | N/A | Trunk, extremities | 14/22 primary | 5/22 FS | Locally unresectable (10/22) and metastatic (12/22) |
N/A |
Rutkowski et al | 2017 | 31 | 56 (20 – 71) | 14 / 17 | N/A | Trunk, head, extremities | 7/31 primary | 16/31 FS | Locally unresectable (16/31) and metastatic (15/31) |
N/A |
Stacchiotti et al | 2015 | 10 | 53 (49 – 73) | 7 / 3 | N/A | Systemic metastasis | 0/10 primary | 10/10 FS | Metastatic DFSP | Absence of metastasis |
Kerob et al | 2010 | 25 | 42.4 (35.6 – 62.1) | 13 / 12 | 4.5 (2.4 – 16) | Trunk, extremities, head and neck | 20/25 primary | All classic DFSP |
Primary or recurrent DFSP ≥ 2 cm | N/A |
Heinrich et al | 2008 | 12 | N/A | N/A | N/A | N/A | N/A | N/A | Life-threatening malignancy refractory to standard therapy |
N/A |
McArthur et al | 2005 | 10 | 47 (23 – 68) | 5 / 5 | N/A | Trunk, extremities, head and neck, genitalia | N/A | 2/10 FS | Locally advanced (8/10) or metastasic disease (2/10) |
N/A |
Han et al | 2009 | 4 | N/A | N/A | 7.0 (2.4 – 7.3) | Trunk, extremities, head and neck | 2/4 primary | N/A | Significant risk of functional or aesthetic compromise after surgery |
N/A |
Rutkowski et al • EORTC • SWOG |
2010 |
16 8 |
47.4 (23.8 – 69.6) 48.6 (28.9 – 66.1) |
11 / 5 3 / 5 |
11.7 (1.2 – 49) 4.5 (1.9 – 27.9) |
Trunk, extremities, head and neck |
4/16 primary 3/8 primary |
7/16 FS 2/8 FS |
Locally advanced/ metastasic not amenable to surgery or radiotherapy with curative intent (EORTC) or R0 resection not feasible with acceptable cosmetic or functional result | Absence of COL1A1 translocation |
Systematic review | 152 | 49.3 (20 – 73) | 65/71 | 9.9 (1.2 – 49) | 61/130 primary | 44/152 FS |
N/A data not available in the study, FS: fibrosarcomatous.
Study with 16 patients but 2 were excluded for non DFSP diagnosis.