Cancers Staged Using This Staging System
Soft tissue sarcomas of the abdominal and thoracic visceral organs, including extraskeletal Ewing sarcoma of the vagina and sarcomas of the cervix are staged in this chapter.
Cancers Not Staged Using This Staging System
| These histopathologic types of cancer… | Are staged according to the classification for… | and can be found in chapter… |
|---|---|---|
| Desmoplastic small round cell tumor (DSRCT) | Soft tissue sarcoma—unusual histologies and sites | 45 |
| Epithelioid hemangioendothelioma (EHE) | Soft tissue sarcoma—unusual histologies and sites | 45 |
| Inflammatory myofibroblastic tumor (IMT) | No AJCC staging system | N/A |
| Perivascular epithelioid cell tumor (PEComa) | No AJCC staging system | N/A |
| Solitary fibrous tumor (SFT) | Soft tissue sarcoma of the retroperitoneum (or other appropriate anatomic site) | 44 |
| Gastrointestinal stromal tumor (GIST), gastrointestinal stromal sarcoma | Gastrointestinal stromal tumor | 43 |
| Carcinomas of the vulva | Vulva | 50 |
| Carcinomas of the vagina | Vagina | 51 |
| Carcinomas of the uterine cervix | Cervix uteri | 52 |
| Leiomyosarcoma, uterine | Corpus uteri - sarcoma | 54 |
| Leiomyosarcoma, retroperitoneal | Soft tissue sarcoma of the retroperitoneum | 44 |
Summary of Changes
| Change | Details of Change | Level of Evidence |
|---|---|---|
| New staging system | A new staging system for abdominal and thoracic and visceral sarcomas is introduced. | IV |
| Definition of Primary Tumor (T) | A new designation for T category is proposed. The designation of deep versus superficial sarcoma does not make sense for these anatomic sites and is deleted. | IV |
| Code | Description |
|---|---|
| C15.1 | Thoracic esophagus |
| C15.2 | Abdominal esophagus |
| C15.4 | Middle third of esophagus |
| C15.5 | Lower third of esophagus |
| C15.9 | Esophagus, NOS |
| C16.0 | Cardia, NOS |
| C16.1 | Fundus of stomach |
| C16.2 | Body of stomach |
| C16.3 | Gastric antrum |
| C16.4 | Pylorus |
| C16.5 | Lesser curvature of stomach, NOS |
| C16.6 | Greater curvature of stomach, NOS |
| C16.8 | Overlapping lesion of stomach |
| C16.9 | Stomach, NOS |
| C17.0 | Duodenum |
| C17.1 | Jejunum |
| C17.2 | Ileum |
| C17.3 | Meckel diverticulum |
| C17.8 | Overlapping lesion of small intestine |
| C17.9 | Small intestine, NOS |
| C18.0 | Cecum |
| C18.1 | Appendix |
| C18.2 | Ascending colon |
| C18.3 | Hepatic flexure of colon |
| C18.4 | Transverse colon |
| C18.5 | Splenic flexure of colon |
| C18.6 | Descending colon |
| C18.7 | Sigmoid colon |
| C18.8 | Overlapping lesion of colon |
| C18.9 | Colon, NOS |
| C19.9 | Rectosigmoid junction |
| C20.9 | Rectum, NOS |
| C21.0 | Anus, NOS |
| C21.1 | Anal canal |
| C21.2 | Cloacogenic zone |
| C21.8 | Overlapping lesion of rectum, anus and anal canal |
| C22.0 | Liver |
| C22.1 | Intrahepatic bile duct |
| C23.9 | Gallbladder |
| C24.0 | Extrahepatic bile duct |
| C24.1 | Ampulla of Vater |
| C24.8 | Overlapping lesion of biliary tract |
| C24.9 | Biliary tract, NOS |
| C25.0 | Head of pancreas |
| C25.1 | Body of pancreas |
| C25.2 | Tail of pancreas |
| C25.3 | Pancreatic duct |
| C25.4 | Islets of Langerhans |
| C25.7 | Other specified parts of pancreas |
| C25.8 | Overlapping lesion of pancreas |
| C25.9 | Pancreas, NOS |
| C26.0 | Intestinal tract, NOS |
| C26.8 | Overlapping lesion of digestive system |
| C26.9 | Gastrointestinal tract, NOS |
| C33.9 | Trachea |
| C34.0 | Main bronchus |
| C34.1 | Upper lobe, lung |
| C34.2 | Middle lobe, lung |
| C34.3 | Lower lobe, lung |
| C34.8 | Overlapping lesion of lung |
| C34.9 | Lung, NOS |
| C37.9 | Thymus |
| C38.0 | Malignant neoplasm of heart |
| C38.1 | Malignant neoplasm of anterior mediastinum |
| C38.2 | Posterior mediastinum |
| C38.3 | Mediastinum, NOS |
| C38.4 | Pleura, NOS |
| C38.8 | Malignant neoplasm of overlapping lesion of heart, mediastinum, and pleura |
| C47.3 | Peripheral nerves and autonomic nervous system of thorax |
| C47.5 | Peripheral nerves and autonomic nervous system of pelvis |
| C49.3 | Connective, subcutaneous and other soft tissues of thorax |
| C49.4 | Connective, subcutaneous and other soft tissues of abdomen |
| C49.5 | Connective, subcutaneous and other soft tissues of pelvis |
| C51.0 | Labium majus |
| C51.1 | Labium minus |
| C51.2 | Clitoris |
| C51.8 | Overlapping lesion of vulva |
| C51.9 | Vulva, NOS |
| C52.9 | Vagina, NOS |
| C53.0 | Endocervix |
| C53.1 | Exocervix |
| C53.8 | Overlapping lesion of cervix uteri |
| C53.9 | Cervix uteri |
| C58.9 | Placenta |
| C60.0 | Prepuce |
| C60.1 | Glans penis |
| C60.2 | Body of penis |
| C60.8 | Overlapping lesion of penis |
| C60.9 | Penis, NOS |
| C61.9 | Prostate gland |
| C62.0 | Undescended testis |
| C62.1 | Descended testis |
| C62.9 | Testis, NOS |
| C63.0 | Epididymis |
| C63.1 | Spermatic cord |
| C63.2 | Scrotum, NOS |
| C63.7 | Other specified parts of male genital organs |
| C63.8 | Overlapping lesion of male genital organs |
| C63.9 | Male genital organs, NOS |
| C64.9 | Kidney, NOS |
| C65.9 | Renal pelvis |
| C66.9 | Ureter |
| C67.0 | Trigone of bladder |
| C67.1 | Dome of bladder |
| C67.2 | Lateral wall of bladder |
| C67.3 | Anterior wall of bladder |
| C67.4 | Posterior wall of bladder |
| C67.5 | Bladder neck |
| C67.6 | Ureteric orifice |
| C67.7 | Urachus |
| C67.8 | Overlapping lesion of bladder |
| C67.9 | Bladder, NOS |
| C68.0 | Urethra |
| C68.1 | Paraurethral gland |
| C68.8 | Overlapping lesion of urinary organs |
| C68.9 | Urinary system, NOS |
WHO Classification of Tumors
This list includes histology codes and preferred terms from the WHO Classification of Tumors and the International Classification of Diseases for Oncology (ICD-O). Most of the terms in this list represent malignant behavior. For cancer reporting purposes, behavior codes /3 (denoting malignant neoplasms), /2 (denoting in situ neoplasms), and in some cases /1 (denoting neoplasms with uncertain and unknown behavior) may be appended to the 4-digit histology codes to create a complete morphology code.
| Code | Description |
|---|---|
| 8711 | Malignant glomus tumor |
| 8800 | Sarcoma, NOS |
| 8801 | Undifferentiated spindle cell sarcoma |
| 8802 | Undifferentiated pleomorphic sarcoma |
| 8810 | Adult fibrosarcoma |
| 8811 | Myxofibrosarcoma |
| 8815 | Solitary fibrous tumor, malignant |
| 8825 | Inflammatory myofibroblastic tumor |
| 8825 | Low-grade myofibroblastic sarcoma |
| 8832 | Dermatofibrosarcoma protuberans |
| 8832 | Fibrosarcomatous dermatofibrosarcoma protuberans |
| 8833 | Pigmented dermatofibrosarcoma protuberans |
| 8840 | Low-grade fibromyxoid sarcoma |
| 8840 | Sclerosing epithelioid fibrosarcoma |
| 8850 | Atypical lipomatous tumor |
| 8850 | Liposarcoma, NOS |
| 8852 | Myxoid liposarcoma |
| 8854 | Pleomorphic liposarcoma |
| 8858 | Dedifferentiated liposarcoma |
| 8890 | Leiomyosarcoma (excluding skin) |
| 8901 | Pleomorphic rhabdomyosarcoma |
| 8910 | Embryonal rhabdomyosarcoma (including botryoid, anaplastic) |
| 8912 | Spindle cell/sclerosing rhabdomyosarcoma |
| 8920 | Alveolar rhabdomyosarcoma (including solid, anaplastic) |
| 9040 | Synovial sarcoma, NOS |
| 9041 | Synovial sarcoma, spindle cell |
| 9043 | Synovial sarcoma, biphasic |
| 9120 | Angiosarcoma of soft tissue |
| 9133 | Epithelioid hemangioendothelioma |
| 9136 | Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma |
| 9136 | Retiform hemangioendothelioma |
| 9180 | Extraskeletal osteosarcoma |
| 9251 | Giant cell tumor of soft tissues |
| 9364 | Extraskeletal Ewing sarcoma |
| 9540 | Malignant peripheral nerve sheath tumor |
| 9542 | Epithelioid malignant peripheral nerve sheath tumor |
| 9561 | Malignant Triton tumor |
| 9580 | Malignant granular cell tumor |
Histology is not ideal for clinical use in patient care, as it describes an unspecified or outdated diagnosis. Data collectors may use this code only if there is not enough information in the medical record to document a more specific diagnosis.
Fletcher CDM, Bridge JA, Hogendoorn P, Mertens F, eds. World Health Organization Classification of Tumours of Soft Tissue and Bone. Fourth Edition. Lyon: IARC; 2013.
International Agency for Research on Cancer, World Health Organization. International Classification of Diseases for Oncology. ICD-O-3-Online.http://codes.iarc.fr/home. Accessed August 16, 2017. Used with permission.
Sarcomas of the abdominal and thoracic viscera represent a varied and heterogeneous group of mesenchymal neoplasms. Traditional staging algorithms have not been able to reliably prognosticate this cohort of sarcomas. This chapter provides a brief introduction to this unique group of sarcomas and proposes a new T classification system for purposes of future data collection and potential development of a specific visceral sarcoma staging algorithm.
Soft tissue sarcomas arise across a breadth of anatomic sites and tissues of origin, and display a variety of different behaviors. This chapter focuses specifically on those arising from abdominal and thoracic viscera.
Soft tissue sarcomas may arise within the abdominal and thoracic cavities, as well as within the solid and hollow visceral organs therein.
Soft tissue sarcomas, including but not limited to leiomyosarcoma and undifferentiated pleomorphic sarcoma, may arise from hollow viscera, including the esophagus (very rare; often presenting as polypoid intraluminal masses with poor prognosis), stomach, small intestine, colon, and rectum, as well as solid viscera such as the liver, kidneys, lungs, and heart.
Soft tissue sarcomas also may arise from the peritoneal or pleural surfaces or in the mediastinum.
The typical mechanism of spread is local extension within the organ or within the involved cavity and hematogenously to distant sites.
Prior staging systems do not apply to sarcomas of visceral organs or to those of the peritoneal and pleural cavities.
Involvement of regional lymph nodes by soft tissue sarcomas is uncommon in adults. Specific histologies in which regional lymph node metastatic disease is most commonly observed include alveolar rhabdomyosarcoma, embryonal rhabdomyosarcoma, epithelioid sarcoma, and angiosarcoma.
Tumors suspected of being sarcoma but without overt malignant features should be classified as having uncertain malignant potential.
As is recognized in other anatomic sites in which there is apparent multifocality, soft tissue sarcomas that present as true multifocal disease (but not metastatic tumors) are challenging to stage. Histologies that may present with true multifocal disease within the abdominal viscera or liver include DSRCT, EHE, and retroperitoneal well-differentiated/dedifferentiated liposarcoma. However, the presence of multifocality is not necessarily of prognostic value for all subtypes.
A new T classification is proposed for visceral sarcomas. Sarcomas arising within the peritoneal, pleural, or mediastinal cavities but not from a specific organ may be staged in a manner similar to that of retroperitoneal sarcomas.
Common sense suggests the number of sites of disease at presentation impacts outcome. With recurrences of some diagnoses, such as well differentiated/dedifferentiated liposarcoma, it is clear that having more than one site of recurrence is worse than a single site of recurrence, since this diagnosis is one that is usually treated surgically. For other diagnoses, such as epithelioid hemangioendothelioma, the utility of the multifocality designation is less clear. Furthermore, there are no criteria by which to declare multifocality vs metastatic disease. This is a determination that will be made clinically. For example, a dominant lesion with small implants elsewhere should be considered metastatic disease, whereas lesions without a dominant primary site can be considered multifocal. Multifocality data will be captured as part of the new staging system to allow further clinical research to continue into this thorny question.
Generally, the extent of disease may be evaluated before treatment with contrast-enhanced computed tomography (CT) of the chest, abdomen, and pelvis. Magnetic resonance imaging may be helpful in specific clinical situations, such as fixed organs, to provider better anatomic definition (e.g., for sarcomas arising in the rectum or liver). Fluorodeoxyglucose positron emission tomography, bone scan, or CT of the head is not routinely necessary for staging but may be used, along with plain films of axial sites, to evaluate areas of ambiguity on other imaging or sites of symptoms.
Accurate histologic classification by an experienced sarcoma pathologist is critical to appropriate care. Reclassification by a sarcoma pathologist has been reported in nearly a quarter of patients seen in a sarcoma specialty clinic, resulting in treatment changes in approximately two thirds of those patients.
At present, there is no role for routine genetic testing for sarcomas arising within abdominal and thoracic viscera. However, selective testing for genetic alterations together with appropriate immunohistochemistry may help distinguish ambiguous cases.
Prognostic Factors Required for Stage Grouping
French Federation of Cancer Centers Sarcoma Group (FNCLCC) grade - see Histologic Grade (G). Where present, multifocality should be documented, as well as the number of involved sites.
Additional Factors Recommended for Clinical Care
There are no additional factors recommended for clinical care.
Presence or absence of mutation in TSC1 or TSC2, evidence of translocation involving TFE3identified in DNA or RNA sequencing of tumor.
The AJCC has recently established guidelines that will be used to evaluate published statistical prediction models for the purpose of granting endorsement for clinical use.2 Although this is a monumental step forward towards the goal of precision medicine, this work was only very recently published. For this reason, the existing models that have been published or may be in clinical use have not yet been evaluated for this cancer site by the Precision Medicine core of the AJCC. In the future, the statistical prediction models for this cancer site will be evaluated, and those that meet all AJCC criteria will be endorsed.
The description of the tumor required for clinical trials varies greatly. For some studies of primary tumors, details of anatomic site and adjoining structures are critical; in studies of metastatic disease, definition of the specific metastatic sites is used for response determination. In nearly all situations, the most detailed definition of the histology is critical—for example, myxoid/round cell liposarcoma instead of liposarcoma—because the biology of each sarcoma subtype is distinct.
Definition of Primary Tumor (T)
| T Category | T Criteria |
|---|---|
| TX | Primary tumor cannot be assessed |
| T1 | Organ confined |
| T2 | Tumor extension into tissue beyond organ |
| T2a | Invades serosa or visceral peritoneum |
| T2b | Extension beyond serosa (mesentery) |
| T3 | Invades another organ |
| T4 | Multifocal involvement |
| T4a | Multifocal (2 sites) |
| T4b | Multifocal (3-5 sites) |
| T4c | Multifocal (greater than 5 sites) |
Definition of Regional Lymph Node (N)
| N Category | N Criteria |
|---|---|
| N0 | No lymph node involvement or unknown lymph node status |
| N1 | Lymph node involvement present |
Definition of Distant Metastasis (M)
| M Category | M Criteria |
|---|---|
| M0 | No metastases |
| M1 | Metastases present |
Definition of Grade (G)
FNCLCC Histologic Grade - see Histologic Grade (G)
| G | G Definition |
|---|---|
| GX | Grade cannot be assessed |
| G1 | Total differentiation, mitotic count and necrosis score of 2 or 3 |
| G2 | Total differentiation, mitotic count and necrosis score of 4 or 5 |
| G3 | Total differentiation, mitotic count and necrosis score of 6, 7, or 8 |
Please see the WHO Classification of Tumors section in this chapter for a list of abdominal and thoracic visceral sarcoma histologies.
HISTOLOGIC GRADE (G)
The FNCLCC grade is determined by three parameters: differentiation, mitotic activity, and extent of necrosis. Each parameter is scored as follows: differentiation (1-3), mitotic activity (1-3), and necrosis (0-2). The scores are added to determine the grade.
Tumor differentiation is histology specific (see chapter 39, table 39.1) and is generally scored as follows:
| Differentiation Score | Definition |
|---|---|
| 1 | Sarcomas closely resembling normal adult mesenchymal tissue (e.g., low-grade leiomyosarcoma) |
| 2 | Sarcomas for which histologic typing is certain (e.g., myxoid/round cell liposarcoma) |
| 3 | Embryonal and undifferentiated sarcomas, sarcomas of doubtful type, synovial sarcomas,soft tissue osteosarcoma, Ewing sarcoma /primitive neuroectodermal tumor (PNET) of soft tissue |
In the most mitotically active area of the sarcoma, 10 successive high-power fields (HPF; one HPF at 400× magnification = 0.1734 mm2) are assessed using a 40× objective.
| Mitotic Count Score | Definition |
|---|---|
| 1 | 0-9 mitoses per 10 HPF |
| 2 | 10-19 mitoses per 10 HPF |
| 3 | Greater than or equal to 20 mitoses per 10 HPF |
Evaluated on gross examination and validated with histologic sections.
| Necrosis Score | Definition |
|---|---|
| 0 | No necrosis |
| 1 | Less than 50% tumor necrosis |
| 2 | Greater than or equal to 50% tumor necrosis |
| G | G Definition |
|---|---|
| GX | Grade cannot be assessed |
| G1 | Total differentiation, mitotic count and necrosis score of 2 or 3 |
| G2 | Total differentiation, mitotic count and necrosis score of 4 or 5 |
| G3 | Total differentiation, mitotic count and necrosis score of 6, 7, or 8 |