|
Stage Information
Nonmetastatic Disease
Metastatic Disease
Recurrent/Progressive Disease
Clinical staging has an important role in predicting the clinical outcome and
determining the most effective therapy for pediatric soft tissue sarcomas. As
yet, there is no well-accepted staging system that is applicable to all
childhood sarcomas; the system from the American Joint Commission for Cancer
(AJCC) that is used for adults has not been validated in pediatric studies, however, the current Children's Oncology Group (COG) trial is using the AJCC staging system (see Table 2 below) to facilitate comparison of results with other pediatric and adult soft tissue sarcoma trials.[1] Two
systems are currently in use for staging pediatric nonrhabdomyosarcomatous soft
tissue tumors. The surgicopathologic staging system used by the Intergroup
Rhabdomyosarcoma Study (see below) is based on the amount of tumor that remains
after initial surgery and whether the disease has metastasized.[2]
Nonmetastatic Disease
-
Group I: Tumor completely resected with histologically negative margins.
-
Group II: Grossly resected tumor with microscopic residual tumor.
-
Group III: Incomplete resection or biopsy with gross residual tumor.
Metastatic Disease
-
Group IV: Any localized or regional tumor with distant metastases present
at the time of diagnosis.
Recurrent/Progressive Disease
- Any soft tissue sarcoma that recurs after initial treatment or progresses after radiation therapy,
chemotherapy, or initial surgery.
The other schema typically used to stage pediatric soft tissue tumors is the
TNM system of the International Union Against Cancer.[3] In
this staging system, T1 lesions are those that are confined to the organ of
origin, and T2 lesions invade adjacent organs. These categories can be
subclassified to reflect the maximum tumor diameter (a: ≤5 cm; b: >5 cm).
Nodal involvement is indicated by N1 (N0: no nodal involvement), and the
presence of distant metastases at the time of diagnosis is indicated by the M1
(vs. M0) designation. Several adult and pediatric series have shown that
patients with large or invasive tumors have a significantly worse prognosis
than do those with small, noninvasive tumors.
These two staging systems have proven to be of prognostic significance in
pediatric and adult nonrhabdomyosarcomatous soft tissue sarcomas (NRSTSs).[4-8] In a
review of a large adult series of nonrhabdomyosarcomas, superficial extremity
sarcomas have a better prognosis than deep tumors. Thus, in addition to grade
and size, the depth of invasion of the tumor should be considered.[9]
Although a standardized staging system for pediatric NRSTS does not exist, the current COG trial is using the sixth edition AJCC staging system for soft tissue sarcomas (with central pathology review) described in Table 2 below.[10]
Table 2. AJCC Staging System for Soft Tissue Sarcomasa
|
|
Primary Tumorb
|
Regional Lymph Nodes
|
Distant Metastasis
|
Histologic Gradec
|
|
Stage I
|
Any tumor size, superfical or deep |
N0 |
M0 |
G1 |
| G2 |
|
Stage II
|
T1a (tumor ≤5 cm in maximal diameter, superficial) |
N0 |
M0 |
G3 |
| T1b (tumor ≤5 cm in maximal diameter, deep) |
N0 |
M0 |
G3 |
| T2a (tumor >5 cm in maximal diameter, superficial) |
N0 |
M0 |
G3 |
|
Stage III
|
T2b (tumor >5 cm in maximal diameter, deep) |
N0 |
M0 |
G3 |
|
Stage IV
|
Any tumor size, superfical or deep |
N1 |
M0 or M1 |
G1, G2, or G3 |
| Any tumor size, superfical or deep |
N0 or N1 |
M1 |
G1, G2, or G3 |
|
G1 = well differentiated; G2 = moderately differentiated; G3 = poorly differentiated; M0 = no distant metastasis; M1 = distant metastasis; N0 = no regional lymph node metastasis; N1 = regional lymph node metastasis
|
|
aAdapted from AJCC Cancer Staging Manual.[10]
|
|
bSuperficial tumor is located above the superficial fascia without invasion of the fascia; deep tumor is located either exclusively beneath the superficial fascia or superficial to the fascia with invasion of or through the fascia. All intraperitoneal visceral lesions, retroperitoneal, pelvic, and intrathoracic tumors, and the majority of head and neck tumors are classified as deep tumors.
|
|
cThe histologic grade established by central pathology review is to be used for staging purposes.
|
References
-
Weiss SW, Goldblum JR: Enzinger and Weiss's Soft Tissue Tumors. 4th ed. St. Louis, Mo: Mosby, 2001.
-
Maurer HM, Beltangady M, Gehan EA, et al.: The Intergroup Rhabdomyosarcoma Study-I. A final report. Cancer 61 (2): 209-20, 1988.
[PUBMED Abstract]
-
Harmer MH, ed.: TNM Classification of Pediatric Tumors. Geneva: UICC, 1982.
-
Rao BN: Nonrhabdomyosarcoma in children: prognostic factors influencing survival. Semin Surg Oncol 9 (6): 524-31, 1993 Nov-Dec.
[PUBMED Abstract]
-
Pisters PW, Leung DH, Woodruff J, et al.: Analysis of prognostic factors in 1,041 patients with localized soft tissue sarcomas of the extremities. J Clin Oncol 14 (5): 1679-89, 1996.
[PUBMED Abstract]
-
Coindre JM, Terrier P, Bui NB, et al.: Prognostic factors in adult patients with locally controlled soft tissue sarcoma. A study of 546 patients from the French Federation of Cancer Centers Sarcoma Group. J Clin Oncol 14 (3): 869-77, 1996.
[PUBMED Abstract]
-
Pappo AS, Fontanesi J, Luo X, et al.: Synovial sarcoma in children and adolescents: the St Jude Children's Research Hospital experience. J Clin Oncol 12 (11): 2360-6, 1994.
[PUBMED Abstract]
-
Pratt CB, Maurer HM, Gieser P, et al.: Treatment of unresectable or metastatic pediatric soft tissue sarcomas with surgery, irradiation, and chemotherapy: a Pediatric Oncology Group study. Med Pediatr Oncol 30 (4): 201-9, 1998.
[PUBMED Abstract]
-
Brooks AD, Heslin MJ, Leung DH, et al.: Superficial extremity soft tissue sarcoma: an analysis of prognostic factors. Ann Surg Oncol 5 (1): 41-7, 1998 Jan-Feb.
[PUBMED Abstract]
-
American Joint Committee on Cancer.: AJCC Cancer Staging Manual. 6th ed. New York, NY: Springer, 2002.
Back to Top
< Previous Section | Next Section > |
