Sarcoma

Overview

Sarcomas are cancers that begin in bone or in the soft tissues, such as tendons, cartilage, muscle and fat, and can be found in any part of the body. This rare type of cancer is more often diagnosed in children, with sarcoma representing approximately 10 to 15 percent of all cancer diagnoses in people under the age of 20. In adults, about 1 percent of all diagnosed solid tumors are sarcoma.

To better understand sarcoma, it helps to have a basic understanding of cancer. Cancer is a collection of many different diseases, and it can develop almost anywhere in the body. Normally, cells divide in an orderly fashion. When they are worn out or damaged, they die, and new cells replace them. Cancer develops when genes begin to change, or mutate, within the structure of normal cells. These cells – now called cancer cells – grow and push against normal cells. If enough cancer cells are created, they may form a mass called a tumor.

Sarcomas are divided into two main groups: soft tissue sarcoma and bone sarcoma.

Soft Tissue Sarcoma

Soft tissue sarcoma can develop in the soft tissues anywhere in the body (see Figure 1). The soft tissues include cartilage, fat, muscle, blood vessels, fibrous tissue and other connective or supportive tissue. Most soft tissue sarcomas occur in the arms or legs.

More common than bone sarcoma, there are more than 50 types of soft tissue sarcoma (see common types in Table 1), and they are named according to where in the body they develop. For example, gastrointestinal stromal tumors (GISTs) develop in the stroma (supportive connective tissue) of the stomach and intestines; sarcomas that develop in fat tissue are called liposarcomas (“lipo” means “fat”); and sarcomas that develop in blood vessels are called angiosarcomas (“angio” means “vessel”).

Table 1. Types of Soft Tissue Sarcoma

Type of Soft Tissue Sarcoma Tissue of Origin Typical Age (Years) Most Common Sites
Most common types
Gastrointestinal stromal tumors
Stroma (supportive connective tissue) of the stomach and intestines
50 and older Wall of the stomach, small intestine
Leiomyosarcoma Smooth muscle tissue Average, 60 Uterus, small intestine or stomach
Liposarcoma Fat tissue 30-60 Thigh, behind the knee, retroperitoneum (behind the abdomen)
Synovial sarcoma Tissue around joints 15-35 Near the foot, ankle, hand
Undifferentiated pleomorphic sarcoma (previously called
malignant fibrous histiocytoma)
Uncertain 50-70
Leg; may also develop in the retroperitoneum (behind the abdomen) or head and neck
Other types
Alveolar rhabdomyosarcoma Skeletal muscle Adolescents and young adults Large muscles of the trunk, arm, leg
Alveolar soft-part sarcoma Connective tissue Young adults Legs or extremities
 
Anaplastic
rhabdomyosarcoma (previously called pleomorphic
rhabdomyosarcoma)
Skeletal muscle Over age 30 Large muscles of the trunk, arm, leg
Angiosarcoma Inner lining of blood vessels 60-70 Skin, breast, liver, spleen, head and neck
Botryoid rhabdomyosarcoma Skeletal muscle Average, 7 Genital region, urinary tract
 
Desmoid tumors
(aggressive fibromatoses)
Connective tissue that forms tendons and ligaments 10-40
Intra-abdominal mesentery (tissue that attaches organs to the wall of the abdomen), arm, leg
Desmoplastic small round cell tumor
Connective tissue that forms tendons and ligaments Adolescents and young adults Abdomen
Embryonal
rhabdomyosarcoma
Skeletal muscle Under age 10
Anywhere, but often in head and neck, and around the eye
Epithelioid sarcoma Skin 20-39 Arm, hand, foot
 
Fibrosarcoma Fibrous tissue 35-55 Thigh, knee, arm, trunk
Hemangiodendothelioma
Inner lining of blood vessels
20-40 Soft tissues or internal organs, such as liver or lung
Malignant peripheral nerve sheath tumors
Cells that surround a peripheral nerve (nerves that connect the central nervous system — spinal cord and brain) with other parts of the body
20-50 Upper part of the arm and leg, trunk

Bone Sarcoma

Bone sarcoma is a malignant (cancerous) tumor that starts in a bone (see Figure 2). Bone sarcoma is different from cancer that spreads to bone from another part of the body, such as the breast or lung. That is called metastatic cancer. Following are the three most common types of primary bone sarcoma (see Table 2).

  • Osteosarcoma, also called osteogenic sarcoma, is the most common bone cancer. It begins in bone cells that make new bone tissue. In children and adolescents, osteosarcoma usually develops at the same time as a growth spurt, and it begins near the ends of long bones, such as in the leg (around the knee) and the upper arm. But osteosarcoma can occur in any bone, especially in older adults, and may be associated with hereditary conditions.
  • Chondrosarcoma, the second most common primary bone cancer, develops inside the bone and is made up of cartilage. Cartilage is fibrous tissue mixed with a gel-like substance, making it softer than bone but harder than most tissues in the body. Chondrosarcoma can occur anywhere in the body where cartilage is present and most frequently develops in the pelvis, legs or arms. Chondrosarcoma occurs primarily in older adults.
  • Ewing sarcoma is the most common tumor in the Ewing family of tumors. This rare bone sarcoma occurs more often in children and young adults and is extremely rare after the age of 25. It usually develops in the pelvis and legs (see Pediatric Sarcoma).

Other types of bone sarcoma, such as adamantinoma, chordoma and malignant giant cell tumor, occur in adults, but these tumors are rare.

Table 2. Types of Bone Sarcoma

Type of Bone Sarcoma Tissue of Origin Typical Age (Years) Most Common Sites
Most common types
Chondrosarcoma Cartilage 40 and older Pelvis, leg, arm
Ewing sarcoma Bone 10-20 Pelvis, leg
Osteosarcoma Bone 10-30 Leg (near the knee), upper arm (near the shoulder)
Other types
Adamantinoma Bone 20-50 Lower leg
Chordoma Bone 40-70 Skull base, spine, tailbone
Malignant giant cell tumor of bone Bone 20-60 Knee

Symptoms

Soft tissue and bone sarcomas are accompanied by different symptoms.

For soft tissue sarcoma, symptoms depend on the location and may be very subtle at first. Pain is not typically a common symptom early on; rather, a person may note the presence of a slowly enlarging, painless mass that may or may not be related to a previous injury. Some sarcomas, including synovial sarcoma and various fibrous tumors, can cause pain. Or, if the tumor touches a nerve, it may cause pain as well. An abdominal mass is often found with a soft tissue sarcoma in the retroperitoneal area (the tissue that lines the abdominal wall), and pain occurs in about half of people with a soft tissue sarcoma in this site. People with soft tissue tumors usually found in the stomach or small intestine may have digestive symptoms, such as nausea, vomiting or a loss of appetite. They may also feel full after eating a small amount of food.

Pain around the tumor site that becomes progressively worse followed by swelling is a common symptom of bone sarcoma. If the tumor is in the lower extremities (hip, thigh, knee, leg or foot), it can cause a painful limp. Movement or weight-bearing activities of the involved extremity may make pain worse.

Managing the symptoms related to bone sarcoma is important and often referred to as supportive or palliative care. Many treatments are available to assist in controlling cancer-related pain issues (see Side Effects). Keep in mind that when your pain is lessened, you will rest better and your body may be able to heal more quickly. Don’t hesitate to talk to your doctor or other members of your treatment team about the pain management options available to you.

Additional Resources

 

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