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Childhood Soft Tissue Sarcoma Treatment (PDQ®)

  • Last Modified: 08/25/2014

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Stages of Childhood Soft Tissue Sarcoma



After childhood soft tissue sarcoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body.

The process used to find out if cancer has spread within the soft tissue or to other parts of the body is called staging. There is no standard staging system for childhood soft tissue sarcoma. Two methods that are commonly used for staging are based on the following:

  • Amount of tumor remaining after surgery to remove the tumor; and/or
  • Grade and size of the tumor; and
  • Whether the tumor has spread to the lymph nodes or other parts of the body.

It is important to know the stage in order to plan treatment.

The following procedure may be used in the staging process:

  • Sentinel lymph node biopsy: A sentinel lymph node biopsy is done to check if cancer has spread to the lymph nodes. The sentinel lymph node is removed during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A small amount of a radioactive substance and/or blue dye is injected near the tumor. The radioactive substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. This procedure is used for epithelioid and clear cell sarcoma.

The results of tests and procedures and the initial surgery are used to find out the stage of the soft tissue sarcoma.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

  • Tissue. The cancer spreads from where it began by growing into nearby areas.
  • Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
  • Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

  • Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
  • Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if soft tissue sarcoma spreads to the lung, the cancer cells in the lung are soft tissue sarcoma cells. The disease is metastatic soft tissue sarcoma, not lung cancer.

One method used to stage childhood soft tissue sarcoma is based on how much cancer remains after surgery to remove the tumor and whether the cancer has spread:

Nonmetastatic childhood soft tissue sarcoma

In nonmetastatic childhood soft tissue sarcoma, the cancer has been partly or completely removed by surgery and has not spread to other parts of the body.

  • Group I: The tumor has been completely removed by surgery.
  • Group II: After surgery to remove the tumor, cancer cells remain that can only be seen with a microscope at the edge of the tissue removed and/or in nearby lymph nodes.
  • Group III: After surgery or biopsy, there is tumor remaining that has not spread but can be seen with the eye.

Metastatic childhood soft tissue sarcoma

  • Group IV: The cancer has metastasized (spread from where it started to other parts of the body).

Another method used to stage childhood soft tissue sarcoma is based on the size of the tumor and whether cancer has spread to lymph nodes or other parts of the body.

This staging system is based on the following:

  • The size of the tumor.
  • Whether the tumor has spread to the lymph nodes.
  • Whether the tumor has spread to other parts of the body.
Enlarge
Tumor size compared to everyday objects; shows various measurements of a tumor compared to a pea, peanut, walnut, and lime
Pea, peanut, walnut, and lime show tumor sizes.

Stage I

Stage I is divided into stages IA and IB:

Stage II

Stage II is divided into stage IIA and stage IIB:

Stage III

In stage III, the tumor is either:

Stage IV

In stage IV, cancer has spread to distant parts of the body such as the lungs. The tumor is any grade or size, and may have spread to nearby lymph nodes.