Table 2:
Characteristics of RMS patients developing CNS metastases
| Age*/Sex | Primary site | Stage / Clinical group* | Histology* | Intra-cranial extension* | Cranial neuro-pathy* | Upfront RT-chemo start interval (wk) | Site(s) of CNS relapse | Time to CNS relapse from dx (mo) | Method of CNS relapse detection** | CNS-directed treatment | Follow-up MRI response after RT initiated | Time to death from CNS relapse (mo) |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Parameningeal primary | ||||||||||||
| 2/F | Petrous bone | III/3 | Embryonal | Yes | Yes | 2 | L | 9 | MRI | None | 0.3 | |
| 3/M | Temporal bone | IV/4 | Embryonal | Yes | Yes | 14 | L | 16 | MRI, LP | CSI | Worsened | 3 |
| 2/F | Infratemporal fossa | IV/4 | Embryonal | Yes | No | 22 | L | 9 | MRI | Chemo | 2 | |
| 4/F | Infratemporal fossa | IV/4 | Embryonal | Yes | No | 14 | L+P | 6 | MRI, Bx | None | 0.3 | |
| 7/F | Infratemporal fossa | III/3 | Embryonal | Yes | Yes | 15 | L | 13 | MRI | None | 0.2 | |
| 6/M | Ethmoid sinus | III/3 | Embryonal | Yes | Yes | 3 | L | 10 | MRI, LP | None | 3 | |
| 31/M | Ethmoid sinus | III/3 | Alveolar | No | No | 10 | L+P | 23 | MRI, LP | WBRT | Improved | 5 |
| 14/F | Maxillary sinus | III/3 | Alveolar | Yes | Yes | 17 | L | 7 | MRI, LP | Intrathecal chemo | 0.5 | |
| 24/F | Maxillary sinus | III/3 | Alveolar | Yes | Yes | 17 | L | 16 | MRI | Chemo, CSI | Improved | 11 |
| 33/M | Frontal sinus | III/3 | Alveolar | Yes | No | 15 | L+P | 13 | MRI, Bx | Surgery, CSI, SRS, 8H9 | Improved | 13 |
| 19/M | Paranasal sinus | III/3 | Alveolar | Yes | Yes | 12 | L,P† (6 mo) | 18 | MRI | CSI | Improved | 8 |
| 24/M | Paranasal sinus + orbit | IV/4 | Alveolar | Yes | Yes | 11 | L+P | 10 | MRI | WBRT (partial course) | 0.2 | |
| 29/F | Paranasal sinus | IV/4 | Alveolar | Yes | Yes | 14 | L | 12 | MRI | Chemo, CSI | Worsened | 5 |
| Extremity primary | ||||||||||||
| 1/M | Buttock | III/3 | Alveolar | 16 | P | 12 | MRI | WBRT | Improved | 11 | ||
| 1/F | Anterior thigh | III/3 | Alveolar | 21 | P,L† (6 mo) | 12 | CT, MRI, Bx | Surgery, CSI, WBRT | Stable | 5 | ||
| 20/M | Hand | IV/4 | Alveolar | 15 | L | 11 | MRI | WBRT | 2 | |||
| 17/M | Forearm | IV/4 | Alveolar | 17 | L+P | 22 | MRI, Bx | Surgery, CSI, WBRT, 8H9 | Improved | 18 | ||
| 27/M | Forearm | IV/4 | Alveolar | 14 | L | 10 | MRI, LP | CSI | Improved | 11 | ||
| 22/M | Forearm | IV/4 | Alveolar | 29 | L | 10 | MRI, LP | CSI | Worsened | 5 | ||
| Other primary | ||||||||||||
| 1/F | Spinal canal | IV/4 | Embryonal | (No RT) | L | 1 | MRI, LP | CSI | Stable | 7 | ||
| 4/F | Paraspinal | IV/4 | Alveolar | 17 | P | 21 | MRI, Bx | Surgery, WBRT, chemo, 8H9 (1 dose) | Improved | 50 | ||
| 13/F | Unknown | IV/4 | Alveolar | (No RT) | L | 17 | Clinical | None | 0.1 | |||
| 26/M | Abdominal wall | IV/4 | Alveolar | (No RT) | L | 11 | MRI | None | 0.1 | |||
*
On initial presentation.
**
Only methods for which positive findings were noted are listed.
†
Developed sequential relapse, displayed in order of presentation with duration in between relapses denoted in parenthesis.
L = leptomeningeal relapse, P = parenchymal relapse, L+P = combined relapse at presentation. Bx = brain biopsy, MRI = magnetic resonance imaging, LP = lumbar puncture, CSI = craniospinal irradiation, WBRT = whole brain radiotherapy, SRS = stereotactic radiosurgery, 8H9 = Intra-Ommaya 131I-8H9, wk = weeks, mo = months.