Rhabdomyosarcoma staging

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Shadan Mehraban, M.D.[2]

Overview

According to the Children's Oncology Group, there are four stages of rhabdomyosarcoma based on the location of tumor, size of tumor, involvement of lymph nodes, and metastasis.

Staging of rhabdomyosarcoma

There are two staging classification for rhabdomyosarcoma:^[1]
- Clinical group (CG)
  - Created by intergroup rhabdomyosarcoma study group (IRSG) in 1972
  - Based on spread of tumor cells at diagnosis and remaining disease after surgery
  - Categorized in four groups
- Tumor, node, metastasis system (TNM)
  - Based on tumor size, node involvement, and metastases
  - Categorized in four groups

Clinical group staging^[2]
Stage	Description
I	Contain 13% of all cases Localized region Complete resection No nodal involvement
II	Contain 20% of all cases IIA group: Complete resection with microscopic residual disease/ no nodal involvement IIB group: Complete resection with no microscopic residual diseases/ regional nodal involvement IIC group: Complete resection with microscopic residual disease/ regional nodal involvement
III	Contain 48% of all cases Incomplete resection or biopsy Gross residual disease
IV	Contain 18% of all cases Distant metastases at time of diagnosis

TNM system^[3]
Stage	Description
1	Involvement of orbit, head and neck (except parameningeal), genitourinory (non-bladder, non-prostate), and biliary tract Tumor stage invasion: T1 ( Confined to anatomic site of origin) or T2 ( include extension) Tumor size: a ( ≤5 cm in diameter) or b ( >5 cm in diameter) Regional nodes: It varies from N0 ( not clinically involve), N1 (clinically involved) to Nx (clinical status unknown) Metastases: M0 (no metastases)
2	Involvement of Bladder, prostate, extremity, cranial parameningeal, and other sites ( trunk, retroperitoneum, etc, excluding biliary tract) Tumor stage invasion: T1 ( Confined to anatomic site of origin) or T2 ( include extension) Tumor size: a ( ≤5 cm in diameter) Regional nodes: N0 (not clinically involve) N1 (clinically involved) Metastases: M0 (no metastases)
3	Involvement of Bladder, prostate, extremity, cranial parameningeal, and other sites ( trunk, retroperitoneum, etc, excluding biliary tract) Tumor stage invasion: T1 ( Confined to anatomic site of origin) or T2 ( include extension) Tumor size: a ( ≤5 cm in diameter) or b ( >5 cm in diameter) Regional nodes: When tumor size is a, regional node category would be N1 ( clinically involve); however, if the tumor size is b, regional node category varies from N0 ( not clinically involve), N1 (clinically involved) to Nx (clinical status unknown) Metastases: M0 (no metastases)
4	Involvement could be all sites Tumor stage: T1 ( Confined to anatomic site of origin) or T2 ( include extension) Tumor size: a ( ≤5 cm in diameter) or b ( >5 cm in diameter) Regional nodes: N0 (not clinically involve) N1 (clinically involved) Metastases: Present distant metastases

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References

↑ Crist WM, Garnsey L, Beltangady MS, Gehan E, Ruymann F, Webber B; et al. (1990). "Prognosis in children with rhabdomyosarcoma: a report of the intergroup rhabdomyosarcoma studies I and II. Intergroup Rhabdomyosarcoma Committee". J Clin Oncol. 8 (3): 443–52. doi:10.1200/JCO.1990.8.3.443. PMID 2407808.
↑ Crist W, Gehan EA, Ragab AH, Dickman PS, Donaldson SS, Fryer C; et al. (1995). "The Third Intergroup Rhabdomyosarcoma Study". J Clin Oncol. 13 (3): 610–30. doi:10.1200/JCO.1995.13.3.610. PMID 7884423.
↑ Crist WM, Anderson JR, Meza JL, Fryer C, Raney RB, Ruymann FB; et al. (2001). "Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease". J Clin Oncol. 19 (12): 3091–102. doi:10.1200/JCO.2001.19.12.3091. PMID 11408506.

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[pmid2407808-1] Crist WM, Garnsey L, Beltangady MS, Gehan E, Ruymann F, Webber B; et al. (1990). "Prognosis in children with rhabdomyosarcoma: a report of the intergroup rhabdomyosarcoma studies I and II. Intergroup Rhabdomyosarcoma Committee". J Clin Oncol. 8 (3): 443–52. doi:10.1200/JCO.1990.8.3.443. PMID 2407808.

[pmid7884423-2] Crist W, Gehan EA, Ragab AH, Dickman PS, Donaldson SS, Fryer C; et al. (1995). "The Third Intergroup Rhabdomyosarcoma Study". J Clin Oncol. 13 (3): 610–30. doi:10.1200/JCO.1995.13.3.610. PMID 7884423.

[pmid11408506-3] Crist WM, Anderson JR, Meza JL, Fryer C, Raney RB, Ruymann FB; et al. (2001). "Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease". J Clin Oncol. 19 (12): 3091–102. doi:10.1200/JCO.2001.19.12.3091. PMID 11408506.

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Rhabdomyosarcoma staging

Overview

Staging of rhabdomyosarcoma

References

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