TYPES OF SARCOMA

CLASSIFICATION:  Soft tissue sarcomas are classified according to the type of soft tissue they resemble.  The types of soft tissue sarcoma include:

Tumors of fibrous (connective)tissue:

     -desmoid tumor

     -fibrosarcoma

Fibrohistiocytic tumors:

     -malignant fibrous histiocytoma

Fat tissue tumors:

     -liposarcoma

Smooth muscle tumors:

     -leiomyosarcoma

Blood and lymph vessel tumors:

     -angiosarcoma

     -hemangiopericytoma

     -hemangioendothelioma

Synovial (joint) tissue sarcoma:

     -synovial sarcoma

Peripheral nervous system tumors:

     -malignant schwannoma(malignant peripheral nerve sheath tumor)

Bone and cartilage tumors:

     -extraosseous osteosarcoma

     -extraosseous myxoid chondrosarcoma

     -extraosseous mesenchymal chondrosarcoma

Combination tissue type tumors:

     -malignant mesenchymoma

Tumors of unknown origin:

     -alveolar soft part sarcoma

     -epitheloid sarcoma

     -clear cell sarcoma (malignant melanoma of soft parts)

SOFT TISSUE SARCOMA

CHILDREN:

Rhabdomyosarcoma:  This is a type of cancer that occurs in the muscles, most common of the soft tissue tumors diagnosed in children.  It can appear anywhere in the body, but most commonly originates in the head and neck region; extremities (arms, elbows, legs, knees, thighs, feet, or shoulders; trunk (abdominal wall, breast or chest wall); orbits (bones of the eye socket or optic nerves; pelvis; and gastrointestinal tract.  The disease can occur anytime throughout childhood, but is most commonly diagnosed in children under the age of six.  There is a slightly higher incidence of this type in boys.  Rhabdomyosarcomas are typically classified into four types:

Embryonal - The most common type and is most frequently seen in head and neck and genitourinary tumors.

Botryoid - A variation of the embryonal type that most frequently arises in, polyps, or , grape-like lesions in the genitourinary tract.

Alveolar - This more aggressive form of the disease is most often found in the extremities or trunk.

Pleomorphic - This rarest form of the disease typically occurs in the extremities or trunk.

Overall, more than 60% of patients diagnosed with rhabdomyosarcoma enjoy long-term survival using combination therapies.  For patients diagnosed with early-stage tumors, the cure rate rises to 80% or higher.

ADULTS:

Some of the most common soft-tissue sarcomas occurring in adults include:

Malignant fibrous histiocytoma (MFH), liposarcoma, synovial sarcoma, fibrosarcoma, vascular tumors, epithelioid sarcoma, and clear cell sarcoma.

These can occur in the soft tissues throughout the body.  They are most commonly found in the arms and legs.  Soft-tissue sarcomas in adults are generally treated with radiation and surgery.  Some soft tissue sarcomas such as synovial sarcoma, rhabdomyosarcoma, clear cell sarcoma and epithelioid sarcoma can metastasize to the lymph nodes.

Other Soft Tissue Sarcomas - Desmoid tumor, leiomyosarcoma, angiosarcoma, hemangiopericytoma, malignant Schwannoma, malignant mesenchymoma.

CHILDREN:

Osteosarcoma (Osteogenic) sarcoma:  Osteosarcoma is the most common malignant bone tumor in children and young adults.  It often starts in the ends of the bones where new bone tissue forms as a young person grows.  Bones in the thigh, upper arm, and shin are the most common sites, but osteosarcoma can occur anywhere in the body.  It usually develops during the period of rapid growth during adolescence, as a teenager matures in an adult.  The average age at diagnosis is 15 years.  Osteosarcoma cells can be many different shapes. 

Ewing's sarcoma:  Ewing's sarcoma is the second-most common cancerous bone tumor in children and young adults.  It gets its name from Dr. James Ewing, the doctor who first described the tumor in the 1920's.  It may arise anywhere in the body, but it usually originates in the long bones of the arms and legs, the pelvis, or the chest.  Though more rate, it may also develop in the flat bones of the trunk.  While it is most often a bone tumor, Ewing's sarcoma can develop in the soft tissues near bones as well.  Peripheral primitive neuroectodermal tumors (pPNET) are very rare molecularly-related tumors that often arise outside of the bones.  Treatment would be the same as Ewing's sarcoma.  While Ewing's sarcoma can occur anytime during childhood, it develops most commonly during puberty, when bones are growing rapidly. 

ADULTS:

Chondrosarcoma:  This is the most common bone tumor occurring in adults.  It occurs most often in the pelvis but can occur in any bone and usually in people aged 50 and older. 

Osteosarcoma:  Classic osteosarcoma are high grade and start within the bone.  With time it can destroy the bone and break out into other surrounding soft tissues.

Other bone tumors:  Malignant fibrous histiocytoma or MFH, fibrosarcoma, adamantinoma(which occurs in the tibia), and chordosarcoma(which occurs in the sacrum or base of the skull).