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 that is used for adults has not been validated in pediatric studies.[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]

• 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.

• Group IV: Any localized or regional tumor with distant metastases present at the time of diagnosis.

• 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.[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]

References

1. Weiss SW, Goldblum JR: Enzinger and Weiss's Soft Tissue Tumors. 4th ed. St. Louis, Mo: Mosby, 2001. 
   
   
2. Maurer HM, Beltangady M, Gehan EA, et al.: The Intergroup Rhabdomyosarcoma Study-I. A final report. Cancer 61 (2): 209-20, 1988.  [PUBMED Abstract]
   
   
3. Harmer MH, ed.: TNM Classification of Pediatric Tumors. Geneva: UICC, 1982. 
   
   
4. Rao BN: Nonrhabdomyosarcoma in children: prognostic factors influencing survival. Semin Surg Oncol 9 (6): 524-31, 1993 Nov-Dec.  [PUBMED Abstract]
   
   
5. 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]
   
   
6. 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]
   
   
7. 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]
   
   
8. 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]
   
   
9. 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]
   
   

Back to Top

< Previous Section  |  Next Section >