From: Congenital Rhabdomyosarcoma: a different clinical presentation in two cases
Patient and disease characteristics |
ERMS ptse (n = 16) |
ARMS ptse (n = 17) |
---|---|---|
Gender: female/male (sex ratio) | 5/11 (0.45) | 10/7 (1.4) |
Primary site | ||
PM head & neck | – | 1 |
Non PM head & neck | 6 | 4 |
Orbit | – | – |
GU non bladder-prostate | 2 | – |
GU bladder-prostate | 2 | – |
Extremities | 1 | 7 |
Other site | 5a | 4b |
NA | – | 1 |
IRSG | ||
I | 1 | – |
II | 4 | 1 |
III | 11 | 3 |
IV | 1 | 13c |
Molecular biology | ||
Negative | 2 | 5 |
NA | 12 | 8 |
PAX3-FOXO1 positive | – | 3 |
PAX7-FOXO1 positive | – | – |
Others | 2 [t(2;8)] | 1 [N-myc amplification] |
Therapy | ||
NA | – | 1 |
Surgery only | 2 | – |
Chemotherapy only | 2 | 9 |
Chemotherapy + surgery | 9 | 4 |
Chemotherapy + surgery + radiotherapy | 3 | 2 |
Chemotherapy + radiotherapy | – | 1 |
Outcome | ||
Alive [median months from dg (range)] | 8 [31.5 (6–240)] | 4 [9 (6–240)] |
Dead | 2 | 13d |
NA | 6 | – |