Sarcoma is a rare cancer in adults (1% of all adult cancers), but rather prevalent in children (about 20% of all childhood cancers).  It is made up of many “subtypes” because it can arise from a variety of tissue structures (nerves, muscles, joints, bone, blood vessels – these are collectively referred to as the body’s “connective tissues”).  At any one time, about 50,000 patients and their families are struggling with sarcoma. About 10,000 new cases are diagnosed each year and about 5,000 die each year from sarcoma.

Chondrosarcoma is a rare malignant tumor that produces cartilage matrix. Primary chondrosarcoma is very uncommon, arises centrally in the bone, and is found in children. Secondary chondrosarcoma arises from benign cartilage defects such as osteochondroma or enchondroma. Cartilage is the specialized tissue that serves as a buffer or cushion at joints. Most of the skeleton of an embryo consists of cartilage, which is slowly converted into bone.  This form of cancer often spreads to other areas of the body and can cause life-threatening complications. There are three main types of chondrosarcoma: mesenchymal, clear cell, and dedifferentiated (see also Myxoid and Juxtacortical).



Mesenchymal chondrosarcoma
is an extremely rare, often aggressive form of cancer. Approximately two-thirds of cases of mesenchymal chondrosarcoma affect the bones, especially the spine, ribs or jaws. The remaining cases occur in areas of the body other than bone (extra-skeletal). Unlike conventional chondrosarcomas, mesenchymal chondrosarcomas occur with greater frequency in young adults.

The Randy Miller Story

Clear cell chondrosarcoma is a malignant cartilage tumor that may be the adult variant of chondroblastoma. It is a rare, low-grade tumor with an improved prognosis over other chondrosarcomas. Like chondroblastoma, it is found in the epiphysis of the femur and humerus. Histologically, soft tissue invasion is rare. Clear cell chondrosarcoma has clear cells with vacuolated cytoplasm. The cartilage matrix has significantly calcified trabeculae and giant cells.

Dedifferentiated chondrosarcoma is the most malignant form of chondrosarcoma. This tumor is a mix of low grade chondrosarcoma and high grade spindle cell sarcoma where the spindle cells are no longer identifiable as having a cartilage origin. The dedifferentiated portion of the lesion may have histological features of malignant fibrous histiocytoma, osteosarcoma, or undifferentiated sarcoma. This biphasic quality is evident on x-ray with areas of endosteal scalloping and cortical thickening are contrasted with areas of cortical destruction and soft tissue invasion. Dedifferentiated chondrosarcoma has a 5 year survival of 10%.

Sarcoma Foundation of America

Sarcoma Alliance

Beat Sarcoma

~ by marbleroad on April 23, 2009.

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