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. Author manuscript; available in PMC: 2022 Mar 10.
Published in final edited form as: JAMA Dermatol. 2019 Mar 1;155(3):361–369. doi: 10.1001/jamadermatol.2018.4940

Table 1:

Systematic review of DFSP treated with imatinib: patient demographics and tumor characteristics.

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.