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.
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
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
• how much dead tissue is in the tumor.
The stages of sarcoma are:
• 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
• Stage IB is the same as stage IA except the
primary tumor is larger than 5 cm (T2).
• 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).
• 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).
• 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