Friday

UNDERSTANDING CHONDROSARCOMA

The following questions about Chondrosarcoma are answered in language the average person can understand. Each question is a link. Click on the question to find the answer.

1) What is Chondrosarcoma?

2) What's the difference between healthy bone and Chondrosarcoma?

3) What are the risks of developing Chondrosarcoma?

4) What are the symptoms of Chondrosarcoma?

5) What locations in the body are Chondrosarcoma tumors found?

6) Why is Chondrosarcoma so rare?

7) Where is the best place to go to receive appropriate treatment?

8) What are the methods used to diagnose Chondrosarcoma?

9) What does "tumor staging" mean in Chondrosarcoma?

10) What are the different kinds of Chondrosarcoma?

11) What are the chances for recurrence, remission, cure and survival?

12) What would happen if no surgery is done to remove the Chondrosarcoma tumor?

13) Why are radiation and chemotherapy ineffective for Chondrosarcoma?

14) What are the possible long term effects of Chondrosarcoma Treatment?

15) Where can I find a support group of others diagnosed with Chondrosarcoma?

16) Are there any alternative methods of treatment known to be successful in curing chondrosarcoma?

17) Who is the long term Chondrosarcoma survivor who created this site?

The information provided is from a layperson's understanding. It is important to check out the facts for yourself with your medical care team.

This site is being maintained by a patient who has survived Chondrosarcoma. I am not a physician. I have no medical professional degree. I am an average person, a patient, who has long term survival, and no recurrence of Chondrosarcoma for many years.

I have learned many things along the way about Chondrosarcoma. I am sharing here what I have learned, and providing as much appropriate information as possible to the best of my ability, and understanding in words the average person can comprehend.

It can be very confusing to research Chondrosarcoma on the internet. There are so many kinds of Sarcoma, and one can get confused sorting out the facts. Over the years, some of my doctors have taught me a great deal. I have attended college courses, including pre-med. I have learned how to do appropriate research on how to find authentic, peer reviewed scientific articles on Chondrosarcoma. I hope to save others from having to spend their lives trying to learn about Chondrosarcoma on their own.

If I can help just one person to get through their experience with Chondrosarcoma, then all my years of going through it alone, and never knowing another soul with the same diagnosis, will have been worth it.

The most valuable lesson I learned from my experiences with Chondrosarcoma? It is imperative to ask questions, get answers, then ask more questions and get more answers. Also, go for a second opinion if necessary, with those most qualified to make the diagnosis and treatment.

Doing this bothered me at first. I wasn't used to questioning doctors and insisting on answers, but it was worth it to be a little uncomfortable. After all, it was my life and my health at stake.

*Note: Chondrosarcoma in English, is pronounced ( KAHN - dro - sar - KO - ma )

Saturday

WHAT'S THE DIFFERENCE BETWEEN HEALTHY BONE AND CHONDROSARCOMA?

In order to understand chondrosarcoma, it is useful to know about normal bone tissue.



What is normal bone tissue?


Bone is the supporting framework of the body protecting internal organs. They act as levers and braces for the muscles. Bone itself is very hard and strong. For example: it takes as much as 1200 to 1800 pounds of pressure to break a healthy femur (thigh bone). A medical word for bone is osteo.


New bone is constantly forming, and old bone is constantly dissolving and contains two kinds of cells to do this. The cells that form new bone tissue are called osteoblasts. The cells that dissolve bone are called osteoclasts. Bone is constantly regenerating itself regardless of age. Although slower after age thirty five.

Most bones are not made of solid bone all the way through. Adult bones are made up of three types of tissue called compact, cancellous and subchondral.


What is compact bone tissue?

The hard, outer portion of most bones consists of fibrous tissue which creates the matrix (base) onto which calcium is deposited. This is the compact bone tissue and is the most solid part of the bone because of the calcium build up.


What is cancellous bone tissue?

Cancellous tissue is the spongy part inside the bones that contains bone marrow. Most people think of this part of the bone as "hollow." But, it is not. The marrow of some bones consists only of fatty tissue. The marrow of other bones is a mixture of fat cells and blood-forming cells where they are created and stored until ready to move into the bloodstream. If surgery on the bone is deep enough, it can occasionally cause bleeding resulting in hemorrhage or anemia. There are some other cells in the marrow, too.


What is subchondral tissue?

Subchondral tissue is the smooth bone tissue of the joints where a layer of cartilage covers each end of the bone to cushion the joint movement.


Cartilage consists of a fibrous tissue mixed with a gel-like substance, and does not contain calcium. The only kind of cells in cartilage are known as chondrocytes. A medical word for cartilage is chondro.


What is abnormal bone growth?

Abnormal bone growth can result in bone deformities, lesions or tumors. There are several different types of bone tumors. The names are based on the area of bone or surrounding tissue that is affected, and the kind of cells forming the tumor. Some primary bone tumors are benign (not cancerous).


Benign bone tumors:

Benign bone tumors do not spread to other tissues and organs, and are not life-threatening. Types of benign bone tumors include osteoma, osteoid osteoma, osteoblastoma, osteochondroma, (also known as exostoses), enchondroma, hemangioma, and chondromyxoid fibroma. Sometimes these may be treated by surgery if they cause problems involving surrounding tissue.

There are other types of malignant bone tumors aside from chondrosarcoma that are also sarcomas. Sarcomas are cancers that mostly develop from bone, cartilage, muscle, fibrous tissue, fatty tissue, or nerve tissue and are identified according to which tissue is rapidly growing.

For better understanding of medical words, go to google and type in DEFINE: and add the word you are seeking (yes, use DEFINE: all in caps with the colon afterward, then skip a space and add the word you want defined.)

For example:
DEFINE: Osteoclast

References:



Skeletal System

Cartilage and Bone

Skeletal Maintenance   
Bone Markers

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Thursday

WHAT IS CHONDROSARCOMA?

Chondrosarcoma is a rare form of bone cancer. It develops from normal cartilage which goes through malignant change, or can form within a pre-existing benign tumor called osteochondroma, or enchondroma. People who have chondrosarcoma have a single tumor growth which can vary in size and location.


Chondrosarcoma is common to humans as well as animals. All patients are not the same in how they are affected. The surgical treatment methods of their doctors are not always the same, either. Each case has to be considered on it's own specific circumstances.
Chondrosarcoma can be referred to by various other names too, depending on the type of cells identified by looking at them under a microscope. It is the second most common primary bone cancer.


For example:
Conventional (Central)
Dedifferentiated
Clear Cell
Extra Skeletal Myxoid
Juxtacortical
Mesenchymal
Periosteal
Synovial


It is important to understand the difference between a benign and malignant bone tumor. Benign bone tumors are not sarcomas. Benign bone tumors do not spread to other tissues or organs, and are not life-threatening. They are generally removed by surgery if there are certain symptoms accompanying them. Again, these are not malignant.


Malignant primary bone tumors are so rare that only two/tenths of a percent of all new cancers. According to the American Cancer Society, "in 2008, about 2,380 new cases of cancer of the bones and joints will be diagnosed in the United States." Remember that means all primary cancers of the bone. Chondrosarcoma is just a small part of that count. In comparison to Breast Cancer, which has been stated as nearly 800,000 annually (in 2006) according to the Susan G. Komen website, bone cancers are very rare


The most common type of bone cancer is osteosarcoma, which develops in new tissue inside growing bones. Evidence suggests that Ewing's sarcoma, another form of bone cancer, begins in immature nerve tissue in bone marrow. Osteosarcoma and Ewing's sarcoma tend to occur more frequently in children and adolescents, while chondrosarcoma occurs more often in adults.


Generally, Chondrosarcoma has a better outcome than osteosarcoma. The treatment options for both these cancers are different. Treatment for chondrosarcoma is less invasive than treatment for Osteosarcoma. Treatment for chondrosarcoma commonly only involves surgical removal of the tumor and surrounding tissue.


Recurrence rates for chondrosarcoma are less than osteosarcoma. Recurrence of chondrosarcoma occurs in the same location as the original tumor or very close to it. Recurrence are rarely found in other parts of the body. Metastasis is not considered recurrence.


Chemotherapy and Radiation treatments are more frequently necessary in osteosarcoma. As of May 2009, chemotherapy and Radiation are not effective and will not produce a cure in chondrosarcomas. If clear margins are done in the original surgery, the likelihood is considerably less.  If the tumor is quite large or has an aggressive grade, amputation, partial amputation or limb salvage surgery may be necessary


If the Chondrosarcoma is located in the skull base, Proton Beam Radiation has been very successful in halting further growth along with necessary surgery. Regular radiation is not sufficiently strong, or as targeted to the tumor as Proton Beam radiation is. Sometimes Proton Beam is used in spinal Chondrosarcoma, and recently some attempts are being made with pelvic chondrosarcoma. Still, surgery is necessary.



Keep in mind that the percentage for full recovery in chondrosarcoma patients is much higher than in osteosarcoma. It is absolutely necessary to have frequent follow up x-rays and scans in order to verify there are no recurrences for at least five years and sometimes eight years. (This is up to the discretion of the doctor). Even if there is a recurrence, there are good chances for long term survival.


Chondrosarcoma is NOT the type of bone cancer that spreads from other organs to the bone. That is called metastatic bone cancer which might be located in more than one bone. A typical example is when lung, kidney, liver, breast or other cancer spreads to the bones as part of their metastasis. Examination of the cells of metastatic bone cancer look like the original type of cancer cells, (prostate, or liver, breast etc.)


These are other types of bone cancer which are NOT bone cancers produced from other types of cancer.


Chondrosarcoma
Ewing's Sarcoma
Fibrosarcoma
Malignant Fibrous Histiocytoma
Giant Cell Tumor of Bone
Chordoma
Lymphoma
Leukemia
Multiple Myeloma
Osteosarcoma 


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Wednesday

WHAT ARE POSSIBILITIES OF DEVELOPING CHONDROSARCOMA?

Chondrosarcoma is not contagious. It cannot be passed on to another person by exposure to a chondrosarcoma patient. Although scientists are not certain what causes chondrosarcoma, a number of factors may put a person at increased risk.



Certain hereditary or genetic conditions are a bit more likely to develop Chondrosarcoma than those who do not have these conditions. Ollier's disease, and Maffucci Syndrome are caused by gene mutations and are not hereditary. Multiple Osteochondromatosis (AKA Multiple Exostoses) is hereditary.

People affected by these conditions are more susceptible because they already have existing benign bone tumors (sometimes mistakenly called bone spurs) which have a chance of becoming malignant. People with these conditions, who experience sudden growth spurts, or increases in hormone production, such as pregnancy, have an increased possibility of a primary benign bone tumor changing into a Chondrosarcoma.


There are other rare conditions which are more likely to pre-dispose to Osteosarcoma. But, some cases are also known to have developed Chondrosarcoma. These are the following.


Retinoblastoma, which is a cancer of the eye, often affects children much more than adults.



Li-Fraumeni, a hereditary syndrome, runs in families where a high percentage of different kinds of cancers occur. These families can be tested. If they do so, and learn which family members test positive, they can be followed closely by their physicians. If they develop a cancer, it can be detected earlier than if they do not receive regular check ups.



Rothmund-Thompson syndrome is a rare condition in itself and there are some occasions of an extremely rare occurrence of Chondrosarcoma. 


Paget's Disease, is a non-cancerous condition characterized by abnormal development of new bone cells in which adults are at increased risk for osteosarcoma or chondrosarcoma.


There are Chondrosarcoma patients who do not have any of these above conditions. 


Some researchers have reported that a previous traumatic injury to the bone have been a possible suspected cause for Chondrosarcoma, but so far this idea is not entirely accepted since there is a controversy and no universal agreement among all sarcoma specialists, though there are often medical articles discussing Chondrosarcoma developing at sites of previous healed fracture.



Some evidence exists that environmental exposure can pre-dispose a person to chondrosarcoma. An example of this known possibility is exposure to known carcinogenic chemicals. There is a question of gardening chemicals such as Agent Orange, also known as Dioxin.


People who have previously had radiation are prone to develop Chondrosarcoma, also.


Recently, chromosomes in the genes have been shown to have specific locations where the genetic information for chondrosarcoma resides. Continuing research of the genes and how the proteins encode for them will give tremendous insight into the growth of cells. 


This information is important since chondrosarcoma is a problem with the growth of cells. Understanding the gene and the function of its protein might eventually provide the knowledge leading to better treatment. Some researchers feel there actually may be hopes that genetic manipulations could treat or prevent chondrosarcoma which may be possible in the future.


The gene mapping studies will serve as the basis for the testing of patients at risk for chondrosarcoma. Information from this kind of testing could lead to the prevention of the development of Chondrosarcoma. And it is hoped that physicians could be equipped to perform such tests in the future. 

NOTE!!!
This update of September 26 2012:


A pair of genes have been found that show up inside of chondrosarcoma tumors. Researchers looked in cells from many different chondrosarcoma tumor samples and found that these two genes, called IDH1 and IDH2, were mutated. 

This shows that they are involved with changing healthy cells into malignant ones. 

The genes are in the tumors themselves and have nothing to do with genetic inheritance from ones family. 

This is not the case with all chondrosarcoma tumors, but with many of them. 

Further research will provide the opportunity to create a drug which can target and attack the mutations. 

These mutations also show up in some other rare cancers, too.


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Tuesday

WHAT ARE THE SYMPTOMS OF CHONDROSARCOMA?

The first symptom may be a solid mass or lump. Pain is often a common symptom of chondrosarcoma. Although some patients do not experience pain and their tumor is discovered quite by accident. 

Symptoms of chondrosarcoma may vary depending on the location and size of the cancer. If the mass interferes with a function of the body, it may cause other feelings such as pressure against other organs in the area of location. Tumors that occur in or near joints may cause swelling or tenderness in the affected area and interfere with normal movements and can weaken the bones, occasionally leading to a fracture.Often chondrosarcoma goes undiagnosed or misdiagnosed at first because the symptoms can mimic other conditions. There can be sciatic-like pain if located in the pelvis or thighs. Nerve damage or vascular compromise can occur when a tumor is pressing on them, too.

The primary symptoms of chondrosarcoma do NOT cause symptoms of fever, fatigue, hair loss, weight loss, or night sweats the way that some other cancers do. These type of symptoms may develop if the chondrosarcoma patient goes through chemo or radiation.

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Monday

WHAT LOCATIONS IN THE BODY ARE CHONDROSARCOMA TUMORS FOUND?

Chondrosarcoma can be located in the body anywhere cartilage is found. The adult human skeleton has 202 to 206 bones, most of which have cartilage. Though some locations are more common than others. Most are commonly found in the pelvis, rib cage, arms, and legs.Although any bone can be affected, the long bones (legs, arms) pelvis and shoulder blades are the most common. While the face and skull are generally unaffected, it is not unheard of. Occasionally chondrosarcoma has been found in the spine, fingers or toes. It is extremely rare to find chondrosarcoma in any internal organs. If Chondrosarcoma metastasizes, it most frequently spreads to the lung.

Skull:

If Chondrosarcoma is found in the skull it is usually found in the skull base, beneath the brain, or near the orbit of the eye, or behind the sinus cavity, occasionally in the jaw. 


See this list for doctors qualified to treat chondrosarcoma of the skull.


See this list of Otolaryngologist/Ent Surgeons who can treat chondrosarcoma of the sinus cavity or larynx


Neck:

If Chondrosarcoma is found in the region of the neck, it can be located in the cartilage of the larynx, (hyoid or cricoid) or the cervical spine.

Arms:

Chondrosarcoma is more frequently found in the upper arm, (the humerus). It is less frequently found in the forearm where two bones are located, 9the ulna and the radius). Very seldom is it found in the hand or fingers.

Legs:

Chondrosarcoma can be found in the upper leg, (thigh bone) medically known as the femur. If the tumor is located high up on the femur near the joint of the pelvis, people often mistakenly say the tumor is in the hip. This area is called the proximal femur. The lower part of the thigh bone is called the distal femur.

The part of the leg that is below the knee has two bones in it, called the fibula and tibia. Chondrosarcoma is located here less often than the femur. Again, the upper parts of these bones are called the proximal and the lower part is called the distal. It is very seldom found in the foot.

Spine:
Chondrosarcoma can be located in any of the vertebrae from the neck all the way down to the tail bone.

Trunk of body:

 
There are many bones that make up the trunk of the body. Chondrosarcoma can be found in almost any of them. Some locations are less affected than others.

Scapula (shoulder blade)
Clavicle (collarbone)
Sternum (holds the rib cage together)
Ribs
Pelvis (made up of ilium, ischium, pubis and acetabulum) 



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Sunday

WHAT IS TUMOR STAGING FOR CHONDROSARCOMA?

Once chondrosarcoma is found, biopsy tests will be done to find out if the cancer cells have spread to other parts of the body. This is called staging. The doctor needs to know the stage of the cancer in order to plan treatment.



There are several staging systems for chondrosarcoma, but no single staging system applies to all types of this cancer. The treatment options in this summary are based on whether the cancer has spread or the amount of tumor left after surgery. The general non specific stages of chondrosarcoma are non-metastatic, metastatic, and recurrent.
 
Treatment by stage
If the Chondrosarcoma is stage I or II the treatment option may simply be surgery to remove all of the cancer. Patients may undergo close monitoring with CT scans on a regular basis to be sure the tumor does not place any vital organs in danger, or if complete removal of the tumor is not possible, or if the tumor comes back following surgery. Sometimes a second operation must be done to be sure that all the tumor has been removed, or if the tumor comes back following treatment. No recurrence of tumor after five years is considered to be cured. 


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WHAT ARE THE METHODS USED TO DIAGNOSE CHONDROSARCOMA?

If a patient has symptoms of a chondrosarcoma, the doctor may need to perform a physical examination based on the symptoms described by the patient. If the patient has a firm, mass attached to the underlying bone with pain or tenderness, this may be an indication to suspect Chondrosarcoma. Recent noticeable growth of a lump, pain and swelling are often the most common complaint. Sometimes, however, there is no obvious evidence of pain and the diagnosis can be confused. There is usually no swelling in any adjacent joint, and range of motion is normal unless tumor has grown large enough to interfere. Fracture of the involved bone is rare. However, in some patients this is the first indication that chondrosarcoma may be present. Systemic symptoms can be associated with the size and location of the tumor. For example, pelvic tumor can present with sciatica, numbness or tingling, extra bladder pressure, or bowel problems. Tumor located in the rib cage can cause pressure upon the lungs, heart, liver or stomach.

In order to determine the diagnosis properly, the doctor may order x-rays and other tests such as a CAT scan, MRI or bone scan. One of these tests, alone, is not enough to determine the diagnosis. So, a combination of these tests helps to reveal the size and characteristics of the tumor and adjacent tissue.

The Radiologist may detect changes consistent with Chondrosarcoma which is most often present on x-ray film as ill-defined lesions with a moderate-sized to large soft tissue component and often demonstrating calcified cartilage. Cat Scan, MRI, Bone Scan, and Ultrasound will give further clarification, and definition of the tumor, and/or in order to determine if other organs are involved.

What do Radiologists look for in a bone scan?
(paraphrased from http://gamma.wustl.edu/bs087te143.html)
"Benign Osteochondroma (exostosis) may show normal to dramatically increased bone uptake. Osteochondroma (benign bone tumor) is relatively uncommon in the spine (1-4% occur in the spine and account for 4% of solitary primary spine bone tumors). 

Normal to mild uptake in an exostosis generally excludes malignancy. However, marked uptake is not specific for malignant degeneration. More intense uptake is seen in growing children. Fracture, of course, results in intense uptake. Thus, the role of bone scan in exostosis or multiple hereditary exostosis is questionable.
Thin-slice CT may provide the diagnosis if marrow and cortical continuity can be demonstrated. MRI will show the characteristic cartilaginous cap (intermediate on T1- and high on T2-weighted images). If this cap is greater than 1 to 2 cm in thickness, concern must be raised of chondrosarcoma.

Chondrosarcoma, the second most common malignant spinal primary bone tumor, is a destructive, lytic tumor with a chondroid matrix consisting of "rings and arcs" radiographically. Cortical destruction is always present (excluded by CT in this case). A soft tissue component is common. In bone scan, these produce patchy or homogeneous increased uptake."

According to an article entitled:
Enchondroma and Chondrosarcoma 
By Flemming DJ, Murphey MD
 
"The judicious use of computed tomography, magnetic resonance imaging, and nuclear medicine in conjunction with appropriate clinical data allows the radiologist to establish the correct diagnosis of benign or malignant medullary chondroid lesion in the majority of cases."

There are no blood tests available at this time useful for diagnosing Chondrosarcoma. Blood tests will eliminate the possibility of other kinds of bone cancer.


Once sufficient studies have been done the doctor may also cut out a small piece of tissue and have a pathologist look at it under the microscope to see, if there are any cancer cells. This is called a biopsy.

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Saturday

What are the different types of Chondrosarcoma?

Chondrosarcoma can be classified in some of the following ways depending on location of the tumor in the body, and the type of cells in the tumor, as determined by the Pathologist within about two weeks of the surgery.

* Clear Cell (CCC) is commonly considered to be a low-grade subtype and less than 1% of chondrosarcoma.

* Conventional - (also known as central) is a low-grade conventional malignant cartilage-forming tumor and makes up approximately 90% of all chondrosarcomas

* Dedifferentiated - high grade sarcoma next to a, comprising 6-10% of all chondrosarcomas

* Extraskeletal Myxoid (EMC) is found in the extremities (5%)

* Juxtacortical (2%)

* Mesenchymal (2%)

* Peripheral - found on the surface of bone within the cartilaginous cap of a solitary chondroma. (15%)

* Primary

* Secondary - due to pre-existing benign tumor


The following are other ways in which chondrosarcoma may be described.

1. Nonmetastatic Chondrosarcoma

The cancer is found only in the area where it started and has not spread to other parts of the body.

2. Metastatic chondrosarcoma

The cancer has spread from where it started to other parts of the body, in most cases, the lung.

Patients who have metastatic chondrosarcoma will rarely receive chemotherapy, radiation therapy, and surgery to remove the cancer that has spread to the lungs.

3. Recurrent chondrosarcoma

The cancer has come back (recurred) after it has been treated. It may come back in the area where it started. A recurrence is rarely found in another part of the body.

Treatment for recurrent chondrosarcoma depends on treatment received previously, the original biopsy staging results, the part of the body where the cancer has come back, and general condition of the patient. Amputation is sometimes necessary and life saving.

What is the possibility of recurrence? Once removed, chondrosarcoma can come back later, but may not immediately re-grow to a size large enough to be symptomatic or as noticeable of the original tumor. It is imperative to have follow-up appointments with the doctor in order to keep track of any further re-growth. Follow up should include scans. The distinction between a recurrence in the same location and recurrence in a different part of the body makes an important difference. A recurrence in the same location, or close to the original tumor is considered to be non-metastatic. A recurrence in a different part of body, usually the lungs, is considered metastatic chondrosarcoma, and more serious.

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Friday

What are the chances for full remission, cure and survival?

The chance of recovery, (prognosis) depends on the type, location, and stage of the tumor. The chance of recovery also depends on the age, size of the tumor, stage of development, and general health of the patient.
In the year 2000, about 2,500 new cases of cancer of the bones and joints were diagnosed, and about 1,400 deaths from these cancers were the expected outcome. Primary cancers of bones account for less than 0.2 percent of all cancers.
What are the percentages of cancers that are bone cancers?
Osteosarcoma is the most common primary bone cancer (35% of cases)
followed by chondrosarcoma (26%)
Ewing's tumor (16%)
chordoma (8%)
malignant fibrous histiocytoma/fibrosarcoma (6%)
Several other more rare cancers account for the remainder of cases.
The long term prognosis for people with primary bone cancer varies greatly, depending on the specific type of cancer and how far it has spread. If you have questions about your personal chances of cure of bone cancer, or how long you might survive such a cancer, it is always best recommended to talk with the people who know your unique circumstances best - your Musculoskeletal or Orthopedic Oncology care team.