Table 5.

Summary of the most important published studies on observation management of desmoid tumor patients

Study	Patients (n)	Abdominal/ Extra-abdominal (n)	Primary/Recurrent (n)	Median tumor size (cm)	Median follow-up (months)	Observation alone (n)	PD (n, %)	SD to PR (n, %)	Regression (n, %)	Tumor behavior	Comparison of PFS between observation and other treatments
Bonvalot et al. [12]	112	46/66	112/0	6	76	11	3 (27%)	NR	NR	NR	NR
Fiore et al. [3]	142	48/94	74/68	6	33	83	33 (40%)	47 (57%)	3 (4%)	47% PFS at 60 months	No difference in PFS between observation and chemotherapy
Barbier et al. [13]	26	0/26	11/15	NR	22	26	1 (4%)	24 (92%)	1 (4%)	Median time to stabilization 14 months	NR
Salas et al. [14]	426	120/306	426/0	7	52	27	6 (22%)	16 (59%)	5 (19%)	Median delay to progression 19.7 months	NR
Briand et al. [15]	73	0/73	31/24	7.5	73	55	5 (9%)	42 (76%)	5 (9%)	Median time to stabilization 9 months	NR
Huang et al. [16]	214	104/100	153/61	5.5	45	20	4 (20%)	14 (70%)	2 (10%)	Median delay to progression 15.3 months	NR
Park et al. [17]	47	7/40	47/0	8	36	20	1 (5%)	18 (90%)	1 (5%)	92% PFS at 36 months	Superior PFS in observation compared to surgery
Burtenshaw et al. [18]	213	213/0	195/18	6	54	63	4 (6%)	36 (57%)	23 (37%)	NR	NR
Penel et al. [19]	771	374/397	NR	5.7	32	388	117 (30%)	NR	NR	58% PFS at 24 months	No difference in PFS between observation and surgery
Orbach et al. [20]	154	NR	NR	NR	21	54	32 (59%)	9 (17%)	4 (7%)	NR	No difference in PFS between observation, surgery and chemotherapy
Cassidy et al. [21]	160	84/76	NR	4.7	15	37	10 (27%)	NR	NR	NR	NR
van Broekhoven et al. [22]	91	25/66	NR	NR	16	37	5 (14%)	25 (68%)	2 (5%)	NR	NR
van Houdt et al. [23]	168	76/92	NR	5.9	41	168	60 (36%)	60 (36%)	45 (27%)	NR	NR
Turner et al. [24]	103	42/61	NR	6.2	35	50	21 (42%)	29 (58%)	0	38% PFS at 36 months	NR
de Bruyns et al. [25]	227	89/138	NR	5.4	77	55	13 (24%)	20 (36%)	22 (40%)	71% PFS at 24 months	NR

PD progression disease, SD stable disease, PR partial response, PFS progression-free survival, NR not reported