Research Article

Targeting the FAK-Src Complex in Desmoplastic Small Round Cell Tumors, Ewing Sarcoma, and Rhabdomyosarcoma

Table 1

Patient characteristics of ES, DSRCT, ARMS, and ERMS tumor tissue.

Tumor typeCharacteristicsN (%)

DSRCT (n = 13)GenderMale9 (69)
Female4 (31)
Age at diagnosis<18 y
≥18 y
4 (31)
9 (69)
TranslocationEWSR1-WT113 (100)
MetastasesYes10 (77)
Unknown3 (23)
Initial metastasesYes10 (100)a
Sample sourcePrimary tumor9 (69)
Resection (post-treatment)4 (31)

ES (n = 68)GenderMale31 (46)
Female37 (54)
Age at diagnosis<18 y44 (65)
≥18 y24 (35)
TranslocationEWSR1-FLI156 (82)
EWSR1-ERG9 (13)
Positive (not specified)3 (5)
MetastasesYes19 (28)
No30 (44)
Unknown19 (28)
Initial metastasesYes7 (37)a
Sample sourcePrimary tumor33 (49)
Resection (post-treatment)18 (26)
Metastasis11 (16)
Local recurrence4 (6)
Other2 (3)

ARMS (n = 21)GenderMale13 (62)
Female8 (38)
Age at diagnosis<18 y15 (71)
≥18 y6 (29)
TranslocationPAX3-FOXO110 (48)
PAX7-FOXO15 (24)
Positive (not specified)6 (28)
MetastasesYes9 (43)
No3 (14)
Unknown9 (43)
Initial metastasesYes3 (33)a
Sample sourcePrimary tumor7 (33)
Lymph node7 (33)
Metastasis1 (5)
Resection (post-treatment)3 (14)
Local recurrence3 (14)

ERMS (n = 39)GenderMale35 (90)
Female4 (10)
Age at diagnosis<18 y35 (90)
≥18y4 (10)
MetastasesYes2 (5)
No33 (85)
Unknown4 (10)
Initial metastasesYes1 (50)a
Sample sourcePrimary tumor31 (80)
Resection (post-treatment)6 (15)
Metastasis2 (5)

DSRCT: desmoplastic small round cell tumor; ES: Ewing sarcoma; ARMS: alveolar rhabdomyosarcoma; ERMS: embryonal rhabdomyosarcoma; N: number of patients; a percentage calculation: (total with initial metastases/total with metastases) 100%.