Rhabdomyosarcoma staging: Difference between revisions

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{| class="wikitable"
{| class="wikitable"
|+Clinical group staging<ref name="pmid7884423">{{cite journal| author=Crist W, Gehan EA, Ragab AH, Dickman PS, Donaldson SS, Fryer C et al.| title=The Third Intergroup Rhabdomyosarcoma Study. | journal=J Clin Oncol | year= 1995 | volume= 13 | issue= 3 | pages= 610-30 | pmid=7884423 | doi=10.1200/JCO.1995.13.3.610 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7884423  }} </ref>
|+Clinical group staging<ref name="pmid7884423">{{cite journal| author=Crist W, Gehan EA, Ragab AH, Dickman PS, Donaldson SS, Fryer C et al.| title=The Third Intergroup Rhabdomyosarcoma Study. | journal=J Clin Oncol | year= 1995 | volume= 13 | issue= 3 | pages= 610-30 | pmid=7884423 | doi=10.1200/JCO.1995.13.3.610 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7884423  }} </ref>
!Group
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Group
!Description
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Description
|-
|-
|I
| style="background:#DCDCDC;" align="center" + |I
|
| style="background:#F5F5F5;" + |
* Contain 13% of all cases
* Contain 13% of all cases
* Localized region
* Localized region
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* No nodal involvement
* No nodal involvement
|-
|-
|II
| style="background:#DCDCDC;" align="center" + |II
|
| style="background:#F5F5F5;" + |
* Contain 20% of all cases
* Contain 20% of all cases
* IIA group: Complete resection with microscopic residual disease/ no nodal involvement
* IIA group: Complete resection with microscopic residual disease/ no nodal involvement
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* IIC group: Complete resection with microscopic residual disease/ regional nodal involvement
* IIC group: Complete resection with microscopic residual disease/ regional nodal involvement
|-
|-
|III
| style="background:#DCDCDC;" align="center" + |III
|
| style="background:#F5F5F5;" + |
* Contain 48% of all cases
* Contain 48% of all cases
* Incomplete resection or biopsy
* Incomplete resection or biopsy
* Gross residual disease
* Gross residual disease
|-
|-
|IV
| style="background:#DCDCDC;" align="center" + |IV
|
| style="background:#F5F5F5;" + |
* Contain 18% of all cases
* Contain 18% of all cases
* Distant metastases at time of diagnosis
* Distant metastases at time of diagnosis
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{| class="wikitable"
{| class="wikitable"
|+TNM system<ref name="pmid11408506">{{cite journal| author=Crist WM, Anderson JR, Meza JL, Fryer C, Raney RB, Ruymann FB et al.| title=Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease. | journal=J Clin Oncol | year= 2001 | volume= 19 | issue= 12 | pages= 3091-102 | pmid=11408506 | doi=10.1200/JCO.2001.19.12.3091 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11408506  }}</ref>
|+TNM system<ref name="pmid11408506">{{cite journal| author=Crist WM, Anderson JR, Meza JL, Fryer C, Raney RB, Ruymann FB et al.| title=Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease. | journal=J Clin Oncol | year= 2001 | volume= 19 | issue= 12 | pages= 3091-102 | pmid=11408506 | doi=10.1200/JCO.2001.19.12.3091 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11408506 }}</ref><ref name="pmid27955730">{{cite journal| author=Dasgupta R, Fuchs J, Rodeberg D| title=Rhabdomyosarcoma. | journal=Semin Pediatr Surg | year= 2016 | volume= 25 | issue= 5 | pages= 276-283 | pmid=27955730 | doi=10.1053/j.sempedsurg.2016.09.011 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27955730 }} </ref>
!Stage
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Stage
!Description
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Description
|-
|-
|1
| style="background:#DCDCDC;" align="center" + |1
|
| style="background:#F5F5F5;" + |
* Involvement of orbit, head and neck (except parameningeal), genitourinory (non-bladder, non-prostate), and biliary tract
* 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 stage invasion: T1 ( Confined to anatomic site of origin) or T2 ( include extension)
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* Metastases: M0 (no metastases)
* Metastases: M0 (no metastases)
|-
|-
|2
| style="background:#DCDCDC;" align="center" + |2
|
| style="background:#F5F5F5;" + |
* Involvement of Bladder, prostate, extremity, cranial parameningeal, and other sites ( trunk, retroperitoneum, etc, excluding biliary tract)
* 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 stage invasion: T1 ( Confined to anatomic site of origin) or T2 ( include extension)
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* Metastases: M0 (no metastases)
* Metastases: M0 (no metastases)
|-
|-
|3
| style="background:#DCDCDC;" align="center" + |3
|
| style="background:#F5F5F5;" + |
* Involvement of Bladder, prostate, extremity, cranial parameningeal, and other sites ( trunk, retroperitoneum, etc, excluding biliary tract)
* 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 stage invasion: T1 ( Confined to anatomic site of origin) or T2 ( include extension)
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* Metastases: M0 (no metastases)
* Metastases: M0 (no metastases)
|-
|-
|4
| style="background:#DCDCDC;" align="center" + |4
|
| style="background:#F5F5F5;" + |
* Involvement could be all sites
* Involvement could be all sites
* Tumor stage: T1 ( Confined to anatomic site of origin) or T2 ( include extension)
* Tumor stage: T1 ( Confined to anatomic site of origin) or T2 ( include extension)
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{| class="wikitable"
{| class="wikitable"
|+Risk classification<ref name="pmid11846299">{{cite journal| author=Raney RB, Anderson JR, Barr FG, Donaldson SS, Pappo AS, Qualman SJ et al.| title=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. | journal=J Pediatr Hematol Oncol | year= 2001 | volume= 23 | issue= 4 | pages= 215-20 | pmid=11846299 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11846299  }}</ref>
|+Risk classification<ref name="pmid11846299">{{cite journal| author=Raney RB, Anderson JR, Barr FG, Donaldson SS, Pappo AS, Qualman SJ et al.| title=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. | journal=J Pediatr Hematol Oncol | year= 2001 | volume= 23 | issue= 4 | pages= 215-20 | pmid=11846299 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11846299  }}</ref>
!Risk type
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Risk type
!Description
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Description
|-
|-
|Low
| style="background:#DCDCDC;" align="center" + |Low
|
| style="background:#F5F5F5;" + |
* Patients with embryonal rhabdomyosarcoma at a favorable sites ( stage 1)
* 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 sites with complete resection ( group I)
* Patients with embryonal rhabdomyosarcoma at an unfavorable site with microscopic residual disease (group II)
* Patients with embryonal rhabdomyosarcoma at an unfavorable site with microscopic residual disease (group II)
|-
|-
|Intermediate
| style="background:#DCDCDC;" align="center" + |Intermediate
|
| style="background:#F5F5F5;" + |
* Patients with embryonal rhabdomyosarcoma at an unfavorable site with gross residual disease (group III)
* 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 metastatic embryonal rhabdomyosarcoma and age < 10 years,
* Patients with nonmetastatic alveolar rhabdomyosarcoma
* Patients with nonmetastatic alveolar rhabdomyosarcoma
|-
|-
|High
| style="background:#DCDCDC;" align="center" + |High
|
| style="background:#F5F5F5;" + |
* Patient with metastatic disease (excluding patients with metastatic embryonal rhabdomyosarcoma and age < 10 years)
* Patient with metastatic disease (excluding patients with metastatic embryonal rhabdomyosarcoma and age < 10 years)
|}
|}

Revision as of 17:15, 1 February 2019

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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]
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), 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
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

  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.
  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.
  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.
  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.
  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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