Carli et al listed prognostic factors for pediatric patients with malignant peripheral nerve sheath tumor (MPNST). The authors are from multiple oncology centers in Italy and Germany.
Parameters:
(1) IRS (Intergroup Rhabdomyosarcoma Study) postsurgical grouping system
(2) tumor size in centimeters
(3) tumor invasiveness
(4) neurofibromatosis type 1
(5) location
|
Parameter |
Finding |
Points |
|
IRS group |
I (completely excised) |
0 |
|
|
II (grossly resected but microscopic residual disease or involvement regional lymph nodes) |
0 |
|
|
III (gross residual disease) |
1 |
|
|
IV (metastatic disease at onset) |
1 |
|
tumor diameter |
<= 5 cm |
0 |
|
|
> 5 cm |
1 |
|
tumor invasive |
confined to tissue or organ of origin (TNM 1) |
0 |
|
|
invasion of contiguous structures (TNM 2) |
1 |
|
neurofibromatosis Type 1 |
no |
0 |
|
|
yes |
1 |
|
location |
extremity |
0 |
|
|
head and neck |
1 |
|
|
trunk, visceral, retroperitoneum |
1 |
where:
• The relative risk for each risk factor is approximately 2 if present.
total score =
= SUM(points for all 5 factors)
Interpretation:
• minimum total score: 0
• maximum total score: 5
• The lower the score the better the overall survival and progression-free survival.
Specialty: Hematology Oncology, Surgery, general, Surgery, orthopedic
