American Joint Committee on Cancer • 2006 191
22 Soft Tissue Sarcoma
(Kaposi’s sarcoma, dermatofibrosarcoma protuberans, fibromatosis [desmoid tumor], and sarcoma arising from the dura mater, brain, parenchymatous organs, or hollow viscera are not included.)
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SUMMARY OF CHANGES
• Angiosarcoma and malignant mesenchymoma are no longer included in the list of histologic types for this site.
• Gastrointestinal stromal tumor and Ewing’s sarcoma/primitive neuroectodermal tumor have been added to the list of histologic types for this site.
• Fibrosarcoma grade I has been replaced by fibromatosis (desmoid tumor) in the list of histologic types not included in this site.
• G 1–2, T2b, N0, M0 tumors have been reclassified as Stage I rather than Stage II disease.
C38.0 Heart
C38.1 Anterior mediastinum C38.1 Posterior
mediastinum C38.3 Mediastinum, NOS C38.8 Overlapping lesion of
heart, mediastinum, and pleura C47.0 Peripheral nerves and
autonomic nervous system of head, face, and neck C47.1 Peripheral nerves and
autonomic nervous system of upper limb and shoulder C47.2 Peripheral nerves and
autonomic nervous system of lower limb and hip
C47.3 Peripheral nerves and autonomic nervous system of thorax C47.4 Peripheral nerves and
autonomic nervous system of abdomen
C47.5 Peripheral nerves and autonomic nervous system of pelvis C47.6 Peripheral nerves and
autonomic nervous system of trunk, NOS C47.8 Overlapping lesion of peripheral nerves and autonomic nervous system C47.9 Autonomic nervous
system, NOS C48.0 Retroperitoneum C48.1 Specified parts of
peritoneum C48.2 Peritoneum, NOS C48.8 Overlapping lesion of
retroperitoneum and peritoneum C49.0 Connective, subcuta-
neous, and other soft tissues of head, face, and neck C49.1 Connective, subcuta-
neous, and other soft tissues of upper limb and shoulder
C49.2 Connective, subcuta- neous, and other soft tissues of lower limb and hip C49.3 Connective, subcuta-
neous, and other soft tissues of thorax C49.4 Connective, subcuta-
neous, and other soft tissues of abdomen C49.5 Connective, subcuta-
neous, and other soft tissues of pelvis C49.6 Connective, subcuta-
neous, and other soft tissues of trunk, NOS C49.7 Overlapping lesion of connective, subcuta- neous, and other soft tissues
C49.9 Connective, subcuta- neous, and other soft tissues, NOS
INTRODUCTION
The staging system applies to all soft tissue sarcomas except Kaposi’s sarcoma, dermatofibrosarcoma, infantile fibrosarcoma, and angiosarcoma. In addition, sarcomas arising within the confines of the dura mater, including the brain, and sarcomas arising in parenchymatous organs and from hollow viscera are not optimally staged by this system.
192 American Joint Committee on Cancer • 2006 Data to support this staging system are based on current analyses from mul- tiple institutions and represent the recommendations of an AJCC task force on soft tissue sarcoma. In the era of cytoreductive neoadjuvant treatments, clinical and pathologic staging may be altered in the future. Because pathologic staging drives adjuvant therapy decisions, patients should be restaged after neoadjuvant therapies have been administered.
Histologic type, grade, and tumor size and depth are essential for staging.
Histologic grade of sarcoma is one of the most important parameters of the staging system. Grade is based on analysis of various pathologic features of a tumor, such as histologic subtype, degree of differentiation, mitotic activity, and necrosis. Accurate grading requires an adequate sample of well-fixed tissue for evaluation. Accurate grading is not always possible on the basis of needle biopsies or in tumors that have been previously irradiated or treated with chemotherapy.
The current staging system does not take into account anatomic site.
However, anatomic site is known to influence outcome, and therefore outcome data should be reported specifying site. Generic grouping of site is accepted. The following site groups can be used in reports that include sarcomas arising in tissues other than soft tissues (such as parenchymal organs). Extremity and superficial trunk can be combined; viscera, including all the intra-abdominal viscera, can also be combined. Where enough numbers exist, these can be reported by subdivision into the various components of the gastrointestinal tract. Lung, gastrointestinal, genitourinary, and gynecologic sarcomas should be grouped separately.
Site Groups for Soft Tissue Sarcomas Head and neck
Extremity and superficial trunk Gastrointestinal
Genitourinary Visceral Retroperitoneal Gynecologic Breast
Lung, pleura, mediastinum Other
STAGING OF SOFT TISSUE SARCOMA
Inclusions. The present staging system applies to soft tissue sarcomas. Primary sarcomas can arise from a variety of soft tissues. These tissues include fibrous connective tissue, fat, smooth or striated muscle, vascular tissue, peripheral neural tissue, and visceral tissue.
Regional Lymph Nodes. Involvement of regional lymph nodes by soft tissue sarcomas is uncommon in adults. When present, regional nodal disease has prognostic significance similar to that of visceral metastatic disease.
Metastatic Sites. Metastatic sites for soft tissue sarcoma are often dependent on the original site of the primary lesion. For example, the most common site
American Joint Committee on Cancer • 2006 193
of metastatic disease for patients with extremity sarcomas is the lung, whereas retroperitoneal and gastrointestinal sarcomas often have liver as the first site of metastasis.
DEFINITIONS Primary Tumor (T)
TX Primary tumor cannot be assessed T0 No evidence of primary tumor
T1 Tumor 5 cm or less in greatest dimension T1a Superficial tumor(1)(Figure 22.1) T1b Deep tumor (Figure 22.2)
T2 Tumor more than 5 cm in greatest dimension T2a Superficial tumor(1)(Figure 22.1) T2b Deep tumor (Figure 22.3)
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T1a T2a
£5 cm >5 cm
FIGURE 22.1. T1a is defined as a superficial tumor 5 cm or less in greatest dimension and T2a is defined as a superficial tumor more than 5 cm in greatest dimension.
T1b
£5 cm
FIGURE 22.2. T1b is a deep tumor 5 cm or less in greatest dimension.
194 American Joint Committee on Cancer • 2006 Regional Lymph Nodes (N)
NX Regional lymph nodes cannot be assessed N0 No regional lymph node metastasis N1 Regional lymph node metastasis Distant Metastasis (M)
MX Distant metastasis cannot be assessed M0 No distant metastasis
M1 Distant metastasis Histologic Grade (G)
GX Grade cannot be assessed G1 Well differentiated G2 Moderately differentiated G3 Poorly differentiated
G4 Poorly differentiated or undifferentiated (four-tiered systems only)(2)
STAGE GROUPING
IA T1a N0 NX M0 G1–2 G1 Low
T1b N0 NX M0 G1–2 G1 Low
IB T2a N0 NX M0 G1–2 G1 Low
T2b N0 NX M0 G1–2 G1 Low
IIA T1a N0 NX M0 G3–4 G2–3 High
T1b N0 NX M0 G3–4 G2–3 High
IIB T2a N0 NX M0 G3–4 G2–3 High
III T2b N0 NX M0 G3–4 G2–3 High
IV Any T N1 M0 Any G Any G High or Low
Any T Any N M1 Any G Any G High or Low
NOTES
1. Superficial tumor is located exclusively above the superficial fascia without inva- sion of the fascia; deep tumor is located either exclusively beneath the superficial fascia, superficial to the fascia with invasion of or through the fascia, or both super- ficial yet beneath the fascia. Retroperitoneal, mediastinal, and pelvic sarcomas are classified as deep tumors.
2. Ewing’s sarcoma is classified as G4.
T2b
>5 cm
FIGURE 22.3. T2b is a deep tumor more than 5 cm in greatest dimension.
