Weiss et al recommended a staging protocol for evaluating children and adolescents with rhabdomyosarcoma. These can help determine what additional testing is required based on clinical and pathologic findings. The authors are from multiple Children's hospitals in the United States and participants in the Children's Oncology Group Soft Tissue Sarcoma Committee.
Patient selection: child or adolescent with rhabdomyosarcoma
Outcome: staging of lung, bone and bone marrow
Parameters:
(1) regional lymph node status (N0 if not clinical involved, N1 if clinically involved)
(2) local tumor invasiveness (T1 = confined to anatomic site of origin, T2 = extension and/or fixation to surrounding tissue)
(3) favorable or unfavorable pathology (unfavorable is alveolar histology and/or PAX-FOXO1 fusion)
(4) CT findings in chest (evidence of lung metastases)
|
Nodal Status |
T Stage |
Pathology |
CT Chest |
Metastatic Workup |
|
N0 |
T1 |
NA |
NA |
none |
|
N0 |
T2 |
favorable |
negative |
no further |
|
N0 |
T2 |
favorable |
positive |
bone scan, bone marrow |
|
N0 |
T2 |
unfavorable |
NA |
full |
|
N1 |
NA |
NA |
NA |
full |
In Table 4, the risk of metastases to various sites were outlined.
|
Nodal Status |
T Stage |
Histology |
Lung Disease |
Lung Metastases |
|
N0 |
T1 |
embryonal |
NA |
0.7% |
|
N0 |
T1 |
alveolar |
NA |
0% |
|
N0 |
T2 |
embryonal |
no |
0% |
|
N0 |
T2 |
embryonal |
yes |
100% |
|
N0 |
T2 |
alveolar |
NA |
12% |
|
N1 |
NA |
embryonal |
no |
0% |
|
N1 |
NA |
embryonal |
yes |
100% |
|
N1 |
NA |
alveolar |
NA |
12% |
|
Nodal Status |
T Stage |
Histology |
Lung Disease |
Bone Metastases |
|
N0 |
T1 |
embryonal |
NA |
0% |
|
N0 |
T1 |
alveolar |
NA |
1.6% |
|
N0 |
T2 |
embryonal |
no |
0.6% |
|
N0 |
T2 |
embryonal |
yes |
17.4% |
|
N0 |
T2 |
alveolar |
NA |
13% |
|
N1 |
NA |
embryonal |
no |
3% |
|
N1 |
NA |
embryonal |
yes |
9.5% |
|
N1 |
NA |
alveolar |
NA |
19.6% |
|
Nodal Status |
T Stage |
Histology |
Lung Disease |
Bone Marrow Metastases |
|
N0 |
T1 |
embryonal |
NA |
0% |
|
N0 |
T1 |
alveolar |
NA |
1.6% |
InfoSpecialty: Hematology Oncology, Surgery, general, Surgery, orthopedic |
