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    Non-RMS

IntroductionEtiologyPresentationRoutes of SpreadInvestigationsStagingGeneral ManagementImagingBiopsySurgeryRadiation TherapyChemotherapySynovial sarcomaPNSTEpitheliod SarcomaLiposarcomaAlveolar Soft PartInfantile FibrosarcomaMFHClear Cell SarcomaDFSPPrognosisReferences

 

 

 

 

 

 

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Soft tissue sarcomas have different routes of spread:

• Local
• Lymphatic
  • Unusual in soft tissue sarcomas but can occur in:
    • Epithelioid sarcoma
    • Clear cell sarcoma
    • Malignant fibrous histiocytoma (pleomorphic sarcoma)
    • Ewing sarcoma (extra-osseous)
    • Synovial sarcoma
    • Alveolar soft part sarcoma
• Distant
  • Generally to lungs, but rarely can spread to bone

 

The American Joint Committee on Cancer (AJCC) has a soft tissue sarcoma staging system to describe extent of disease and prognosis using four criteria:

• Tumor size (T)
• Nodal status (N)
• Metastasis (M)
• Grade (G)

In this system:

• Superficial tumor located:
  • Above the superficial fascia without invasion of the fascia
• Deep tumor located:
  • Beneath the superficial fascia
  • Superficial to the fascia with invasion of or through the fascia or
  • Both superficial yet beneath the fascia.

 

AJCC Primary Tumor Staging:

TX	Primary tumor cannot be assessed
T0	No evidence of primary tumor
T1	Tumor less than or equal to 5 cm in greatest dimension
T1a	Superficial tumor less than or equal to 5 cm in greatest dimension
T1b	Deep tumor less than or equal to 5 cm in greatest dimension
T2	Tumor greater than 5 cm in greatest dimension
T2a	Superficial tumor greater than 5 cm in greatest dimension
T2b	Deep tumor greater than 5 cm in greatest dimension

 

AJCC Lymph Node Staging:

NX	Regional lymph nodes cannot be assessed
N0	No regional lymph node metastases
N1	Regional lymph node metastases

 

AJCC Distant Metastases:

M0	No distant metastases
M1	Distant metastases

 

Grading (simplified)

• Several grading systems have been published and correlate with prognosis
• Two most important parameters appear to be mitotic index and extent of tumor necrosis.

 

Grade 1	Tumor closely resembles normal adult mesenchymal tissue
	Low mitotic count
	No necrosis
Grade 2	Sarcomas where histological typing is certain
	Intermediate mitotic count
	<50% tumor necrosis
Grade 3	Embryonal and undifferentiated sarcomas Synovial sarcomas Osteogenic sarcomas
	High mitotic count
	> or = 50% tumor necrosis

 

AJCC Anatomic Stage:

Stage 1A	T1a	N0	M0	G1, GX
	T1b	N0	M0	G1, GX
Stage 1B	T2a	N0	M0	G1, GX
	T2b	N0	M0	G1, GX
Stage IIA	T1a	N0	M0	G3, G3
	T1b	N0	M0	G3, G3
Stage IIB	T2a	N0	M0	G2
	T2b	N0	M0	G2
Stage III	T2a, T2b	N0	M0	G3
	Any T	N1	M0	Any G
Stage IV	Any T	Any N	M1	Any G

 

Link to Staging PDF

 

 

 

References:

WHO classification of soft tissue sarcomas

Stage information for adult soft tissue sarcomas at the NCI

 

 

 

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