Table 1.
Characteristics of RMS xenograft models and response to cycle 1 of VAC therapy
| Model ID | Subclass | Age (years) | Gender | Site | Diagnosis or relapse | Response | EFS (weeks) |
| Rh10 | ARMS | 15 | Female | Liver | Relapse | PD | 6 |
| Rh28 | ARMS | 17 | Male | Hand | Diagnosis | MCR | > 20 |
| Rh30R | ARMS | 16 | Male | Bone marrow | Relapse | CR | 9 |
| Rh41 | ARMS | 12 | Female | Unknown | Autopsy | PD | 3 |
| Rh18 | ERMS | 2 | Female | Perineum | Diagnosis | CR | 10 |
| Rh36 | ERMS | 15 | Male | Paratesticular | Relapse | PR | 6 |
| Rh66 | ARMS | 12 | Female | Axillary lymph node | Metastasis | CR | 7 |
| Rh12 | ERMS | 12 | Male | Right buttock | Diagnosis | MCR | > 20 |
| NCH-ERMS-1 | ERMS | 5 | Male | orbital | No treatment | PD | 5 |
| NCH-ARMS-2 | ARMS, metastatic | 16 | Female | Right breast | Treated | PD | 4 |
| Rh71 | ERMS, high grade | 17 | Male | Prostate | Diagnosis | PD | 6 |
| Rh72 | ERMS | 3 | Female | Perineal | Treated | PD | 9 |
| Rh73 | ERMS | 5 | Male | Right infratemporal fossa mass | Treated | PD | 7 |
| Rh75 | ERMS | 17 | Male | Pelvis recurrent (Rh71) | Treated | CR | 8 |
| Rh78 | ARMS | 1 | Male | Right thigh | No treatment | SD | 8 |
| Rh80 | ERMS anaplastic | 5 | Female | Stomach mass | Treated | PD | 5 |
| Rh81 | ERMS | 9 | Male | Abdominal mass | Treated | PR | 7 |
| Rh82 | ARMS | 3 | Male | Paratesticular | Treated | PR | 10 |
| Rh83 | ERMS | 4 | Male | Left orbital mass | Treated | PR | 14 |
| Rh84 | ARMS | 2 | Male | Upper lip lesion | Treated | SD | 12 |
| Rh85 | ERMS diffuse anaplasia | 5 | Female | Abdominal mass | No treatment | PD | 4 |
| Rh86 | ERMS | 8 | Male | Retroperitoneal mass | Treated | CR | 10 |
| Rh87 | Spindle cell/sclerosing | 6 | Female | Oropharyngeal mass | Treated | PD | 3 |
| Rh88 | ERMS | 10 | Male | Pelvic mass | Treated | CR | 12 |
| IRS-56 | ERMS | 3 | Male | Buttock | Diagnosis | CR | > 20 |
| IRS-68 | ERMS | 13 | Male | Shoulder | Diagnosis | PR | 7 |
| SJRHB011_X | ERMS | 5 | Male | Infratemporal fossa | Recurrent | PD | 4 |
| SJRHB013_X | ERMS | 3 | Female | Perineal/bladder | Recurrent | CR | 13 |
| SJRHB010927_X1 | ERMS | 5 | Female | Parapharyngeal | Diagnosis | PR | 12 |
| SJRHB000026-X1 | ERMS | 4 | Female | Pelvis | Recurrent | PD | 5 |
| SJRHB013758_X1 | ERMS | 4 | Female | Abdomen/pelvis | Diagnosis | CR | 10 |
| SMS-CTR* | ERMS | 1 | Male | Pelvis | Diagnosis | MCR | > 20 |
| JR-1 (UK)* | ERMS | 7 | Female | Lung | Relapse | PD | 7 |
| RD* | ERMS | 7 | Female | Pelvis | Relapse | SD | 11 |
*Indicates cell line derived xenograft. ARMS: Fusion-positive; ERMS: fusion negative; RMS: rhabdomyosarcoma; VAC: vincristine/actinomycin-D/cyclophosphamide; EFS: event-free survival; PD: progressive disease; MCR: maintained complete response; CR: complete regression; PR: partial response; SD: stable disease.