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CARTILAGE CANCER

What Are the Normal Soft Tissues?

The human body is made up of individual cells, of which there are many various kinds. Initially,when the fathers sperm and mothers egg join to form a fertilized egg, each cell in the fledglinghuman embryo is exactly the same. As the embryos cells divide, the genetic material withinthem, called DNA, instructs certain cells to specialize - that is become heart cells, lung cells,bone cells, brain cells, etc. This process of specialization is called differentiation, and accountsfor the wide variety of cells making up adults. The early embryo separates cells into 3 basicdistinctions - cells for making skin, brain and nerves (ectoderm), cells for making the organs(endoderm), and cells cells for making bone, flesh and blood (mesoderm). However, ultimatelythe mesoderm cells are found in all parts of the growing human.

Certain specialized cells are programed to form tissues, which are a collection of cells for a

certain purpose. Examples of tissues include skin, muscle, fat, bone, blood and cartilage. Thetissues are joined in complex patterns to form organs, which contain many types of cells. Forinstance, the ear is an organ which contains bone (the ossicles), muscle fibers, nerves, fat,cartilage and skin, all precisely organized. Organs are further arranged into organ systems. Theear must be properly connected to another organ, the brain, to function. Other examples of organsystems include the skeletal, digestive, urinary, and reproductive systems. Still, as complex as thesystems become, they still are made of single cells as the smallest unit of life.

The mature human body contains organs, bones, flesh, and blood, and we said that each of thesecomplex structures are formed from simpler tissues. The soft tissues of the body are basically itsflesh - formed of muscles, fat, cartilage, and fibrous tissues. These tissues are not only found as

the middle layer (mesoderm) between the skin and bone, but are also a part of many internalorgans, such as the stomach, bladder, and uterus, and bone. Thus, a disease of the soft tissues canactually arise almost anywhere in the body! This is because these basic tissues are the buildingblocks of nearly all a humans parts. While the bone is initially from the mesoderm flesh (it startsout as cartilage), as it becomes calcified it is no longer considered a soft tissue (after all it ishard!). Thus, diseases, including cancer, of bone a classified separately from soft tissue diseases,even though the same tissues may be involved. Skin is also classified separately since it arisesfrom a different area of the developing embyro (ectoderm), along with the nerves and brain.

Thus, the basic soft tissues making flesh in the adult are the muscle, fat, fibrous tissues (sinews),cartilage, and blood vessels. To understand disease of these, we must look at them on their

cellular level. There are actually three types of muscle cells in the body, several types of fibrouscell, but only one type of fat and cartilage cell. The blood vessels are made up of layers ofvarious types of cells. For muscle cells, there are intercalated heart cells (not discussed here)striated skeletal muscle cells, and smooth organ muscle cells. The diseases from them are namedin Latin:

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**Chondro - means arising from cartilage cells. In a growing bone, the cells lay down cartilagewhich is formed of various condroitin proteins. The proteins also called "glucose-amino-glycans"or "GAGs"; they are made of chains of amino acids linked by sugars. Later, the cartilage canbecome calcified to form hard bone, but other areas (ears, nose, joints, ribs) will contain cartilagethroughout adult life. Cartilage is nourished by diffusion of tissue fluid.

Rhabdomyo - means arising from the striated, or skeletal muscle. This type of muscle shows asmall patterns of bands (striations) under the light microscope, and is under voluntary control -the muscles we can move such as in the face and limbs. Muscles often have specific vessels thatfeed them.

Leiomyo - (Lie-Oh-My-Oh) means arising from the smooth muscles. This type of muscle occursas linings within organs, arranged in circular or longitudinal patterns. It is not under voluntarycontrol, but instead controlled by the autonomic nervous system - by the body itself. Examples ofthis kind of muscle are contracting linings of the intestines, uterus, and blood vessels.

Lipo - means arising from fat cells, called adipocytes. These fat cells are found in all sorts ofbody areas, including behind the eyeballs , within the bone marrow and in the liver. Most fatcells are in the trunk and limbs.

Fibro - means arising from the fibrous tissues (sinews) of the body, and in particular thefibroblasts which are the basic scar tissue of the body.

Synovial - a specialized type of fibrous tissue making the sacs that surround certain joints, likethe knees. These cells secrete special fluid to lubricate.

Neurofibro - means arising from the linings around nerves, made up of an insulating substancecalled myelin (its what makes nerves white). The myelin is produced by Schwann cells, whichline the nerves and are also considered a fibrous soft tissue. Only the nerve linings are a softtissue.

Angio, Hemangio - means arising from the blood vessel cells. While blood is not considered asoft tissue, the blood vessels are.

Osteo - means arising from bone. Even though bone tumors are considered separately from softtissue tumors, the bone is a mesenchymal (middle layer) tissue and may be found mixed intoother soft tissue tumors.

Meso or Myxo - this means arising from a mixture of soft tissues, which may be blood vessels,cartilage, fat, fibrous tissues, and smooth or striated muscle. It is related to Mesothelium (middlelayer) also called Mesenchymal.

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You can see the range of crucial soft tissues in the body, and understanding the above prefixesallows one to categorize the diseases that arise from these mesechymal cells. It is not uncommonfor more that one type of cell to be involved with a disease process, so sometimes the names areconjoined together to describe the cells seen under the microscope. Examples include

Chondrofibro, Osteochondro and Lipofibro; they are all mesenchymal (middle embryo layer)diseases.

How Common is Soft Tissue Sarcoma?

Each year in there about 8,000 new cases of Soft Tissue Sarcoma in the United States, whichcause approximately 2,500 deaths per year. Thus, they represent ~1% of all new cancers. SoftTissue Sarcoma is about 3 times more common than Bone Sarcoma. There are two peaks of mostcommon patient age, one in childhood at 10 years old and the other in 40 year old adults. ThusSarcomas are unlike the other major type of cancer, Carcinomas (e.g. breast, lung, prostate, colo-

rectal) which all tend to get more likely as we age. Males and Females are overall equallyaffected by Sarcomas. In children, the most common type of Soft Tissue Sarcoma isRhabdomyosarcoma (from skeletal muscle cells), which occur mostly in the Head and Neckareas. In contrast to childhood cases, the least common area for adult sarcomas is in the Headand Neck area. In adults, the most common type is now Fibrosarcoma or the closely relatedMalignant Fibrous Histiocytoma, which tends to occur in the trunk or extremities. These account(in some series) for up to 80% of soft-tissue sarcoma cases. "Chondrosarcoma" is the secondmost common sarcoma, with about 500 cases per year in the U.S.A. Overall, the number of newcases of Soft Tissue Sarcoma has remained stable for the past 3 decades.

What Causes, or Increases the Risk for Getting a Sarcoma?

As with every cancer, the precise reason why one person gets a Sarcoma and another does notremains unknown. This is particularly true for Chondrosarcoma, which often arises with nopreceding risks identified. However, depending upon the particular subtype, various things havebeen noted (by studying groups of patients) which seem to increase overall sarcoma risk:

1) Carcinogens (Chemical Exposure) - Some chemicals have the ability to get into cells anddamage their DNA, leading to disease. The chemical may kill cells, leading to an increase inproduction of new ones, but some cells may not be killed only injured. These cells often try hardto repair themselves, but may try to divide before succeeding with complete repair. The more

often damaged cells try to divide, the higher the chance for a cancer to arise. Some chemicalsshown to raise the risk for cancer in general, and sarcomas in particular, include Dioxin,Polyvinyl Chloride, Arsenic, Benzene, and Thorium.

2) Radiation Exposure - Ionizing radiation passes through cells and damages their DNA. Thismay lead to malfunction of genes which would normally prevent the damaged cell from dividing(suppressor gene), or turn on a potential cancer gene (oncogene). The most common type of

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cancers arising from prior radiation treatment are sarcomas. Early watch makers licked theirradium-tipped brushed (used to pain luminous dials), getting bone sarcomas.

While the risk is extremely low with occasional diagnostic X-rays, getting X-ray therapy(especially for a cancer caused by a genetic disease {e.g. retinoblastoma} may result in a 20%

risk of later sarcomas (radiation carcinogenesis).

3) Viruses - These actually get into cells, insinuate themselves into the DNA, and start givingtheir own instructions to the cell! In the process, they can do damage to the host DNA, leading tocancer. Kaposis sarcoma linked with the HIV (AIDS) virus is the best known example.

4) Genetic Diseases (Family Syndromes) - These include disease where the genes are damagedfrom birth, and thus susceptible to turning cancerous (such as Neurofibromatosis, Beck withWeidemann and Retinoblastoma). Also, there are inborn diseases where DNA repair is impaired,or the stop signal that prevents damaged cells from dividing is broken (Li-Fraumeni). With

Chondrosarcoma, there may be preceding Enchondromas (bone cysts) from the rare Maffucci'ssyndrome or Ollier's syndrome.

5) Chronic Irritation - This can stimulate cells to divide, and the more often division occurs(especially in cells that dont normally divide often) the more likely a cancerous one is to arise.Examples include the lymphangiosarcomas seen in limbs with chronic swelling (edema) calledthe Stewart-Treves syndrome in women with arm swelling after treatment for breast cancer. Also,there is more risk for sarcomas around permanent stabilization pins placed into bones and jointsafter traumatic injuries.

The common underlying factor is damage to DNA which causes the affected cell to becometransformed - that is lose control over its division. Cancer is ultimately a disease of the DNA!The DNA is packed into thousands of genes, which are themselves located upon the 48chromosomes (46 general plus 2 sex chromosomes) that all healthy humans have in every cell.The chromosomes become visible under an ordinary light microscope when cells divide, andnearly every case of Sarcoma shows chromosome damage. This damage usually includes piecesmissing from chromosomes (deletions), or even parts of one chromosome getting stuck ontoanother (translocations). Overall, anything which can damage DNA, the fundamental geneticmaterial, will increase the risk of a cell turning cancerous. This damage may be latent, meaning acancer may arise many years or decades after the damage occurs.

Can Chondrosarcomas Be Prevented?

There is no sure way to prevent any sarcoma. It is always a good idea to avoid unnecessaryexposure to potential carcinogens and avoid unwarranted X-rays. This is especially true forpatients with family susceptibility to cancers, or who actually have genetic diseases. If aworrisome sign or symptom arises (see below) it should be evaluated promptly, and not ascribedto some benign process without proof. Eating a diet with enough vitamin C (Ascorbic Acid) is

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important for proper maintenance and healing of soft tissues, especially cartilage. Vitamin Cdeficiency results in a breakdown of the soft tissues (scurvy); it is essential for crosslinking theircrucial collagen proteins. However, taking too much can cause the blood to become too acidic(ascorbic acidemia). A standard supplement multi-vitamin is well advised.

The cartilage in the human frame gives structural support and acts as a barrier to the spread ofinfections. In a small child, the majority of the bones are soft cartilage which gradually "ossifies"with calcium deposits to become adult bone. Cartilage is normally a maintenance-free tissuewhich requires a little blood, some vitamin C and mild exercise to stay healthy. Rarely, a cancerarises from a single abnormal cartilage cell which divides out of control. This type of cancer iscalled "chondrosarcoma". If this cell is to later produce bone, a mixed cartilage-bone cancer myarise. These are called "osteochondrosarcomas" and their therapy is the same as for bonesarcomas.

It is crucial to get prompt diagnosis and proper treatment for a Cartilage Cancer. This can meanthe difference between keeping or losing a limb, or even between life and death. Understandingyour options is dealing with a Cartilage Cancer problem will give you the peace of mind ofknowing you have done everything possible for a happy outcome.

Our material explains, in plain English, the definition, types, frequency, risk factors, symptoms,evaluation, historic and latest effective treatment for Cartilage Cancers. We tell you everythingyou must know to make the right choices today to deal with a Cartilage Cancer problem.