Pediatric Cancer Care

Pediatric Sarcomas: Other Types of Sarcoma

In general, sarcomas that are not classified as osteosarcoma, Ewing sarcoma, rhabdomyosarcoma, or desmoplastic small round cell tumors are rare in children, adolescents, and young adults.

Treatment for these soft tissue sarcomas is tailored to each patient, and can include a combination of chemotherapy, surgery, and radiation therapy.

These tumors are classified by the tissue in which they originate, including:

Fibrohistiocytic Tumors

Malignant fibrous histiocytoma (MFH), also called spindle cell sarcomas

Tumors of Adipose Tissue

Liposarcoma

Tumors of Bone and Cartilage

Extraosseous osteosarcoma
Extraosseous myxoid chondrosarcoma
Mesenchymal chondrosarcoma

Tumors of Fibrous Tissue

Fibromatoses (desmoid tumors)
Fibrosarcoma
Dermatofibrosarcoma
Low-grade fibromyxoid sarcoma

Tumors of the Gastrointestinal Tract

Gastrointestinal stromal tumors (GIST)

Tumors of More Than One Tissue Type

Malignant mesenchymoma
Malignant triton tumor
Malignant ectomesenchymoma

Tumors of the Peripheral Nervous System

Malignant schwannoma (malignant peripheral nerve sheath tumor)

Tumors of Smooth Muscle

Leiomyosarcoma

Tumors of Unknown Origin

Alveolar soft part sarcoma (ASPS)
Clear cell sarcoma (malignant melanoma of soft parts)
Epithelioid sarcoma
Synovial sarcoma
Undifferentiated soft tissue sarcoma

Tumors of Vascular Structures

Angiosarcoma
Hemangioendothelioma
Hemangiopericytoma
Lymphangiosarcoma

alveolar soft part sarcoma (al-VEE-oh-ler … sar-KOH-muh)

A soft tissue tumor that is most common in older children and teenagers. It begins in the soft supporting tissue that connects and surrounds the organs and other tissues. Alveolar soft part sarcoma usually occurs in the legs, but can also occur in the arms, hands, head, or neck. It can cause the growth of new blood vessels that help the tumor grow and spread. Also called ASPS.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

cell (sel)

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

chemotherapy (KEE-moh-THAYR-uh-pee)

Treatment with drugs that kill cancer cells.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

clear cell (kleer sel)

A type of cell that looks clear inside when viewed under a microscope.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

desmoplastic (DES-moh-PLAS-tik)

Causing or forming adhesions or fibrous connective tissue within a tumor.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

Ewing sarcoma (YOO-ing sar-KOH-muh)

A type of cancer that forms in bone or soft tissue. Also called peripheral primitive neuroectodermal tumor and pPNET.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

extraosseous (EK-struh-AH-see-us)

Located outside of the bone.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

fibrous (FY-brus)

Containing or resembling fibers.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

gastrointestinal (GAS-troh-in-TES-tih-nul)

Refers to the stomach and intestines. Also called GI.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

malignant (muh-LIG-nunt)

Cancerous. Malignant cells can invade and destroy nearby tissue and spread to other parts of the body.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

melanoma (MEH-luh-NOH-muh)

A form of cancer that begins in melanocytes (cells that make the pigment melanin). It may begin in a mole (skin melanoma), but can also begin in other pigmented tissues, such as in the eye or in the intestines.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

mesenchymal (meh-ZEN-kih-mul)

Refers to cells that develop into connective tissue, blood vessels, and lymphatic tissue.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

nerve (nerv)

A bundle of fibers that receives and sends messages between the body and the brain. The messages are sent by chemical and electrical changes in the cells that make up the nerves.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

osteosarcoma (OS-tee-oh-sar-KOH-muh)

A cancer of the bone that usually affects the large bones of the arm or leg. It occurs most commonly in young people and affects more males than females. Also called osteogenic sarcoma.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

radiation (RAY-dee-AY-shun)

Energy released in the form of particle or electromagnetic waves. Common sources of radiation include radon gas, cosmic rays from outer space, medical x-rays, and energy given off by a radioisotope (unstable form of a chemical element that releases radiation as it breaks down and becomes more stable).

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

rhabdomyosarcoma (RAB-doh-MY-oh-sar-KOH-muh)

Cancer that forms in the soft tissues in a type of muscle called striated muscle. Rhabdomyosarcoma can occur anywhere in the body.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

sarcoma (sar-KOH-muh)

A cancer of the bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

schwannoma (shwah-NOH-muh)

A tumor of the peripheral nervous system that arises in the nerve sheath (protective covering). It is almost always benign, but rare malignant schwannomas have been reported.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

soft tissue (... TIH-shoo)

Refers to muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

surgery (SER-juh-ree)

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

therapy (THAYR-uh-pee)

Treatment.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

tissue (TIH-shoo)

A group or layer of cells that work together to perform a specific function.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)

undifferentiated (un-DIH-feh-REN-shee-AY-ted)

A term used to describe cells or tissues that do not have specialized ("mature") structures or functions. Undifferentiated cancer cells often grow and spread quickly.

Source: The National Cancer Institute's Dictionary of Cancer Terms
(http://www.cancer.gov/dictionary)