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Childhood Soft Tissue Sarcoma Treatment (PDQ®): Treatment - Patient Information [NCI] - General Information About Childhood Soft Tissue Sarcoma

Childhood soft tissue sarcoma is a disease in which malignant (cancer) cells form in soft tissues of the body.

Soft tissues of the body connect, support, and surround other body parts and organs. The soft tissues include the following:

  • Fat.
  • A mix of bone and cartilage tissue.
  • Fibrous tissue.
  • Muscles.
  • Nerves.
  • Tendons (bands of tissue that connect muscles to bones).
  • Synovial tissues (tissues around joints).
  • Blood vessels.
  • Lymph vessels.

Soft tissue sarcoma may be found anywhere in the body. In children, the tumors form most often in the arms, legs, or trunk (chest and abdomen).

There are many different types of soft tissue sarcomas.

The cells of each type of sarcoma look different under a microscope. The soft tissue tumors are grouped based on the type of soft tissue cell where they first formed.

This summary is about the following types of soft tissue sarcoma:

Fat tissue tumors

  • Liposarcoma. This is a rare cancer of the fat cells. There are several different types of liposarcoma. Most liposarcomas in children and teenagers are low grade (likely to grow and spread slowly). The most common type is myxoid liposarcoma, which is usually low grade and responds well to treatment. Another type is pleomorphic liposarcoma, which is usually high grade (likely to grow and spread quickly) and is less likely to respond well to treatment.

Bone and cartilage tumors

Bone and cartilage tumors are a mix of bone cells and cartilage cells. Bone and cartilage tumors include the following types:

  • Extraskeletalchondrosarcoma. This type of bone and cartilage tumor includes mesenchymal chondrosarcoma, which grows quickly and often spreads to the lungs.
  • Extraskeletal osteosarcoma. This type of bone and cartilage tumor is very rare in children and teenagers. It is likely to come back after treatment and may spread to the lungs.

Fibrous (connective) tissue tumors

Fibrous (connective) tissue tumors include the following types:

  • Desmoid tumor (also called aggressive fibromatosis). This fibrous tissue tumor is low grade (likely to grow slowly). This tumor may come back in nearby tissues but usually does not spread to distant parts of the body. Rarely, the tumor may disappear without treatment.

    Desmoid tumors sometimes occur in children with changes in the adenomatous polyposis coli (APC) gene. Changes in this gene cause familial adenomatous polyposis (FAP). FAP is an inherited condition in which many polyps (growths on mucous membranes) form on the inside walls of the colon and rectum. Genetic counseling (a discussion with a trained professional about inherited diseases and a possible need for gene testing) may be needed.

  • Fibrosarcoma.

    There are two types of fibrosarcoma in children and teenagers:

    • Infantile fibrosarcoma (also called congenital fibrosarcoma). This type of fibrosarcoma is found in children aged 4 years and younger. It most often forms in infants and may be seen in a prenatal ultrasound exam. This tumor is often large and fast growing, but rarely spreads to distant parts of the body. The cells of this tumor usually have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). In order to diagnose infantile fibrosarcoma, the tumor cells are checked for this genetic change.
    • Adult-type fibrosarcoma. This is the same type of fibrosarcoma found in adults. The cells of this tumor do not have the genetic change found in infantile fibrosarcoma.
  • Dermatofibrosarcoma protuberans. This is a rare fibrous tissue tumor found in children and adults. The cells of this tumor have a certain genetic change called a translocation. In order to diagnose dermatofibrosarcoma protuberans, the tumor cells are checked for this genetic change.
  • Inflammatory myofibroblastic tumor. This is a fibrous tissue tumor that is likely to come back after treatment but rarely spreads to distant parts of the body. A certain genetic change has been found in about half of these tumors.
  • Low-grade fibromyxoid sarcoma. This tumor may come back many years after treatment and is likely to spread to the lungs and the lining of the wall of the chest cavity. Lifelong follow-up is needed.
  • Myxofibrosarcoma, low-grade. This is a rare fibrous tissue tumor that is found less often in children than in adults. It is likely to grow slowly.
  • Sclerosing epithelioid fibrosarcoma. This is a rare fibrous tissue tumor that usually grows slowly.
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