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Rhabdomyosarcoma

 

TNM Staging

 

Biopsy is needed to establish the diagnosis for primary - but not for metastatic sites (masses seen in liver or lung) except for bone marrow.

Staging prior to treatment requires

  •  Clinical examination
  • Imaging studies

Pre-treatment size is part of the staging

  • Measured on MRI or CT depending on location.
  • Examination and CT can be used to assess lymph size.
  • Metastatic sites will require some form.

Primary Tumor: TNM Staging of Rhabdomyosarcoma

T1 = Confined to anatomic site of origin

- a. ≤ 5 cm in diameter in size

- b. > 5 cm in diameter in size

T2 = Extension and/or fixative to surrounding tissue

- a. ≤ 5 cm in diameter in size

- b. > 5 cm in diameter in size.

Regional Nodes

N0  =  Regional nodes not clinically involved

N1 =  Regional nodes clinically involved by neoplasm

Nx = Clinical status of regional nodes unknown (especially sites where lymph node evaluation is not possible.

Metastasis

M0 = No distant metastasis

M1 =  Distant metastasis present 

TNM Staging depends on the tumor SITE.

Stage 1

- Head and neck (excluding parameningeal) GU

- Orbit

- Genitourinary  (non-bladder/non-prostate) Biliary Tract

- Biliary Tract

- In addition these tumors must also be T1 or T2 (a or b), N0/1/x and M0

Stage 2  

  • Bladder / prostate
  • Extremity
  • Cranial parameningeal
  • Other: trunk, retroperitoneum, etc

- In addition these tumors must also be T1 or T2 a (not b), N0 or Nx (not N1) and M0

Stage 3  

  • Bladder/Prostate
  • Cranial parameningeal
  • Other: trunk, retroperitoneum, etc

- In additon these tumors must also be

      • T1 or T2
      • If a (≤5cm) then also N1
      • If b (>5cm) then any nodal status (N0/1/x)
      • M0

Stage 4:

  • All sites

 - These tumors are M1 (distant metastases) with either T1  or 2, a or b and any nodal status (N0/1/x)

TNM Staging Summary

 

Stage 1

Orbit, Head and neck (excluding parameningeal) GU – non-bladder/non-prostate Biliary Tract

T1 or T2 (a or b)

N0 or N1 or Nx

M0

Stage 2  

Bladder/Prostate Extremity, Cranial Parameningeal, Other (includes trunk, retroperitoneum, etc.) Except Biliary tract

T1 or T2 a

N0 or Nx

M0

Stage 3  

Bladder/Prostate

T1 or T2 a

N1

M0

Extremity Cranial Parameningeal, Other(includes trunk,retroperitoneum, etc.) Except Biliary tract

T1 or T2b

 N0 or N1 or Nx

M0

Stage 4

All sites

T1 or T2 a or b

N0 or N1

M1

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