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NCCN Guidelines for Patients


Soft Tissue Sarcoma, Version 1.2014


spread to the bones. Examples of bones include the

spine and pelvis. If you have these sarcomas, you

may receive an MRI of total spine to assess if the

cancer has spread.

Imaging of central nervous system

Alveolar soft-part sarcomas and angiosarcomas are

more likely to spread to the brain. This is especially

true for stage IV alveolar soft part sarcomas that have

spread to the lungs. If you have these sarcomas, you

may receive imaging scans of your central nervous

system. The central nervous system includes your

brain and spinal cord.

Genetic assessment

If you may have Li-Fraumeni syndrome, your doctor

may want you to have a genetic assessment. A

genetics counselor will explain what the goals,

benefits, and risks of the assessment are. The

counselor may also ask more questions about your

health and your family’s health history. You may decide

to get tested for Li-Fraumeni syndrome. To be tested,

you must provide a sample of body tissue. Using the

tissue sample, a pathologist can test your genes for the

TP53 mutations that cause Li-Fraumeni syndrome.

Treatment by stage

The treatment options for sarcomas in this chapter

are listed by stage. Cancer staging is a rating by

your doctors of the extent of the cancer. It is used to

plan which treatments are best for you. The AJCC







ommittee on


ancer) staging

system is used to stage sarcoma. There are four

stages—I, II, III, and IV.

In this system, the letters T, N, and M describe

a different area of cancer growth. The T score

describes the growth of the primary tumor. The N

score describes spread of cancer growth to lymph

nodes. The M score tells if the cancer has spread to

distant sites. The T, N, and M scores are combined to

assign the cancer a stage.

Another factor used in staging is the cancer grade.

Higher-grade sarcomas tend to grow and spread

faster than lower-grade sarcomas. A three-grade

system is used for sarcoma based on scores of:

• how much the cancer cells look like normal


• how fast the cancer cells are making copies of

themselves, and

• how much dead tissue is in the tumor.

The stages of sarcoma are:

Stage I

• Stage IA tumors are 5 cm or smaller (T1).

They can be superficial (T1a) or deep (T1b).

The cancer has not spread to lymph nodes

(N0) or more distant sites (M0). Stage IA

tumors are low grade (G1) or the grade is

unknown (GX).

• Stage IB is the same as stage IA except the

primary tumor is larger than 5 cm (T2).

Stage II

• Stage IIA tumors are 5 cm or smaller (T1),

have not spread (N0, M0), and are either

intermediate (G2) or high (G3) grade.

• Stage IIB tumors are larger than 5 cm, have

not spread, and are intermediate grade (G2).

Stage III

• The primary tumor is larger than 5 cm (T2),

has not spread (N0, M0), and is high grade

(G3). Stage III tumors can also be of any size

or grade and spread to lymph nodes (N1, M0).

Stage IV

• These sarcomas can be of any size or grade

and have spread to distant sites (M1).


Sarcomas in limbs, outer trunk, head, or neck Treatment by stage