Rhabdomyosarcoma staging

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

Overview

There are two staging classification for rhabdomyosarcoma including Children group (CG) and tumor, node, and metastasis system (TNM). Each of them has four stages of rhabdomyosarcoma based on the location of tumor, size of tumor, involvement of lymph nodes, and metastasis. Also, risk stratification system is used for classification of patients into low, intermediate, and high risk groups.

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, and metastasis system (TNM)
  - Based on tumor size, node involvement, and metastases.
  - Categorized in four groups.

Clinical group staging^[2]
Group	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]^[4]
Stage	Description
1	Involvement of orbit, head and neck (except parameningeal), genitourinary (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, extremities, 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, extremities, 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.

Risk classification^[5]
Risk type	Description
Low	Patients with embryonal rhabdomyosarcoma at a favorable sites ( stage 1). Patients with embryonal rhabdomyosarcoma at an unfavorable sites with complete resection ( group I). Patients with embryonal rhabdomyosarcoma at an unfavorable site with microscopic residual disease (group II).
Intermediate	Patients with embryonal rhabdomyosarcoma at an unfavorable site with gross residual disease (group III). Patients with metastatic embryonal rhabdomyosarcoma and age < 10 years. Patients with nonmetastatic alveolar rhabdomyosarcoma.
High	Patient with metastatic disease (excluding patients with metastatic embryonal rhabdomyosarcoma and age < 10 years).

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.
↑ Dasgupta R, Fuchs J, Rodeberg D (2016). “Rhabdomyosarcoma”. Semin Pediatr Surg. 25 (5): 276–283. doi:10.1053/j.sempedsurg.2016.09.011. PMID 27955730.
↑ Raney RB, Anderson JR, Barr FG, Donaldson SS, Pappo AS, Qualman SJ; et al. (2001). “Rhabdomyosarcoma and undifferentiated sarcoma in the first two decades of life: a selective review of intergroup rhabdomyosarcoma study group experience and rationale for Intergroup Rhabdomyosarcoma Study V.” J Pediatr Hematol Oncol. 23 (4): 215–20. PMID 11846299.

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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.

[pmid27955730-4] Dasgupta R, Fuchs J, Rodeberg D (2016). “Rhabdomyosarcoma”. Semin Pediatr Surg. 25 (5): 276–283. doi:10.1053/j.sempedsurg.2016.09.011. PMID 27955730.

[pmid11846299-5] Raney RB, Anderson JR, Barr FG, Donaldson SS, Pappo AS, Qualman SJ; et al. (2001). “Rhabdomyosarcoma and undifferentiated sarcoma in the first two decades of life: a selective review of intergroup rhabdomyosarcoma study group experience and rationale for Intergroup Rhabdomyosarcoma Study V.” J Pediatr Hematol Oncol. 23 (4): 215–20. PMID 11846299.

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Rhabdomyosarcoma staging

Overview

Staging of rhabdomyosarcoma

References

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