Surgical Pathology Criteria–

Rhabdomyosarcoma

Staging and Clinical Risk Groups

Clinical Risk Groups classify patients into low, intermediate and high risk groups based on:

Pathologic Staging is performed using the Pretreatment TNM Staging System established by the Intergroup Rhabdomyosarcoma Study Group (Note: this is not the TNM system described in the AJCC Cancer Staging Manual).

Classification Description
Tumor  
T1 Confined to site of origin
  T1a Tumor size < 5 cm
  T1b Tumor size ≥ 5 cm
T2 Extension to / infiltration of surrounding tissue
  T2a Tumor size < 5 cm
  T2b Tumor size ≥ 5 cm
Regional Lymph Nodes  
N0 Lymph nodes not clinically involved
N1 Lymph nodes clinically involved
NX Clinical lymph node status unknown
Metastasis  
M0 No distant metastasis
M1 Distant metastasis present

Clinical Staging is performed using the Intergroup Rhabdomyosarcoma Pretreatment Clinical Staging System based on Pathologic TNM (above), Site and Size:

Stage Site T Tumor size N M
Stage 1 Favorable T1 or T2 Any N0, N1, NX M0
Stage 2 Unfavorable T1 or T2 < 5 cm N0, NX M0
Stage 3 Unfavorable T1 or T2 < 5 cm N1 M0
    OR ≥ 5 cm N0, N1, NX M0
Stage 4 Any T1 or T2 Any N0, N1 M1
Favorable sites are biliary tract, orbit, head and neck (excluding parameningeal) and genitourinary (excluding prostate and bladder); all others are unfavorable

Clinical Group is determined using the Intergroup Rhabdomyosarcoma Clinical Grouping System based on extent of disease:

Group Extent of Disease
Group I Localized disease, excised
     Group Ia Confined to site of origin
     Group Ib Infiltrative, beyond site of origin; negative lymph nodes
Group II Total gross resection with regional disease spread
     Group IIa Localized tumor with microscopic residual disease
     Group IIb
  • Regional disease with positive lymph nodes, excised
  • No microscopic residual disease
     Group IIc
  • Regional disease with positive lymph nodes
  • Grossly resected with microscopic residual disease
Group III Gross residual disease
     Group IIIa Localized or regional disease, Biopsy
     Group IIIb Localized or regional disease, Resection (debulking of more than 50% of tumor)
Group IV Distant metastasis

Clinical Risk Group is determined using the Children's Oncology Group Stratification for Rhabdomyosarcoma based on the above determined group and stage:

Histologic Subtype

Clinical Group

Clinical Stage

Clinical Risk Group

Embryonal

I, II, III

1

Low risk

Embryonal

I, II

2,3

Low risk

Embryonal

III

2,3

Intermediate risk

Embryonal

IV

4

High risk

Alveolar

I, II, III

1,2,3

Intermediate risk

Alveolar

IV

4

High risk

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