Monday

FAMILY HISTORY OF HEREDITARY BENIGN BONE TUMORS

My Grandfather was a Blacksmith at the turn of the century. Attending to the needs of various farmers, he shoed horses, mended plows, and repaired sleds, wagons, and other farm equipment. After marrying my grandmother, he then took his Blacksmithing skills to the lumber companies throughout the southern tier of New York State, where he and my grandmother, both, became employed. She was camp cook and laundress. He Smithed all logging equipment. By the time my mother was born, the years of hard physical labor had begun to take their toll, and her father retired to a little community to have his own blacksmith shop near the house. Because of his Multiple Hereditary Exostoses he was a man of short stature, with well-muscled, but bowed arms. He walked with a slight limp caused by a troubling lump on an ankle. He died when my mother was nine years old. She inherited her Multiple Exostoses from him, as many of the descendants of my grandfather's side of the family have MHE.

During my mother's childhood she was aware of the strange lumps that she and her brother had. Her mother used to rub liniment on them, to help make the pain and spasms go away. But, her mother didn't know what they were and tried to make light of them, and called them "calcium deposits". Genevieve learned at an early age that folks didn't discuss their medical deformities; they were a forbidden subject, and she learned to hide hers. She and her brother were the only ones she knew who had this deformity. She didn't realize her cousins had them, until she was an adult.

The deformities made my mother feel too different from others. She was ashamed to wear a bathing suit. As a teen, she often tried to get excused from attending her gym class because she didn't want people to notice her deformities. Then one day the teacher had the class do exercises on the floor. My mother was unable to get up, and out of the position they were in. She was totally mortified. The gym teacher had to help her get up. After that, the teacher gave permission for her to skip classes often.

Yet, I know she never grew taller than (What?), five foot four at the most. In her last decades she lost bone density. Yes,keep in mind that even MHE patients can have Osteoporosis. There is no extra calcium in MHE bones, as myth would have it.

My favorite photo of my mother was taken when she was about fourteen. She is perched atop an old-fashioned cabinet style radio. One would never noice that she had such a problem with her legs. She was terribly embarrassed by the lumps on her bones. A particularly large one, which grew slightly below her knee bothered her the most. She was careful to keep it concealed beneath appropriately lengthened skirts.

Unfortunately, one time, a man was noticed it and rudely said enough to speak out loud. "What is that thing on your leg, there, Girlie?". Kids didn't get much respect back then, I guess. She took his comment to heart from that day forward, and felt there was something really "wrong" with her, like she was a "freak," and belonged in a circus sideshow. It marked her emotionally for a long time.
THE NEXT FOUR GENERATIONS
When my older brother was born it didn't occur to my mother that her child would have the "Calcium Deposits", too This was all we knew to call them, we thought that we had too much calcium in our bones. Thank heavens we didn't know too much about foods containing calcium or we probably would have done without them. Chondromas or exostoses are tumors made of cartilage arising from the bone. There is another kind of benign bone tumor called an enchondroma, but they start from within the bone. By the time my older brother was nine, my mother realized he had those bumpy bones. Upon a visit to a doctor, she was told that he should be taken to an Orthopedic Specialist and have the bone bumps surgically removed, but not until after he was twelve. My mother was not told the name of the condition or what to expect from it. That's just how it was in those days. However, the doctor had called it "congenital," and it was the first time my mother began to understand it to be a hereditary condition. That was when she began to feel guilty about passing it on to her four children. Every mother asks herself if they have done something wrong when their children have an illness.

The reality is that there was nothing she could have done to prevent us from having this genetic condition. This does not mean that anyone with MHE will have all children born with MHE. Right now the projected percentage is 50-50. It is called auto-somal dominant. A person with MHE has a 50 percent chance of producing a child with the same gene. It didn't occur to her until much later, that her father had passed it on to her.

My older brother never had the surgeries. He felt he could get by without any. Yet, he suffers pain and mobility difficulties every day. I recall that he had such pain in his legs that he created a wedge to put into his shoe to change the positioning of his foot so that he could walk more comfortably, as one leg was shorter than the other. While riding a long distance with him in his car one time, I noticed that he bounced his knees up and down as he drove. He glanced at me, and said, "My legs get so numb and painful, I have to keep them moving just to have some relief."

All I could say was, "Yeah, I know. I have to do the same thing." I have discovered that it is due to the fact that I have a long piece of tumor growing on the underside of my thigh bone, (femur) and driving puts pressure on it, causing the pain to increase, and bouncing my legs around helps to relieve it. The same thing happens when I go to the movies or sit in a hard chair.

My older brother has a daughter. She has two kids, and now grandkids. I don't know if any of them have MHE.

The first time I saw my baby brother, I noticed a large lump just below his right knee. Since I was just six years old at the time, I thought he actually had been born with three knees. Among the four of us, he was the only one who had such obvious signs at birth. He has had many more exostoses located throughout his body than anyone else in the family except my son. My younger brother had curved forearms like our grandfather (called Madelung's Deformity) He later had surgery and they straightened out pretty well. He tells me that he didn't know he had these bone tumors until he was about seven or eight years old. It was about this time that other kids pointed out his deformities and cruelly teased him. It was then that he refused to wear shorts, no matter what the weather. He also refused to go swimming or participate in sports. With the large amount of bone tumors on his body, it was very difficult to compete with his more agile peers, and he was not too happy to be known to have them. By the age of fifteen a very talented orthopedic surgeon performed surgery on both his legs and an arm for a total of nine tumors. This was the first surgery anyone in our family had experienced. The following year both legs were operated on, at the joints, to remove five more osteochondromas. My younger brother has since grown a taller and his legs have NOT gotten knock-kneed like the rest of us. He can move and bend and physically do things the rest of us cannot. He is badly scarred, though. But, he doesn't mind.

I consider the scars like "Battle Scars". Any soldier who goes off to war and suffers such scarring usually receives a medal. So does my brother! And anyone else who goes through MHE surgeries to make their lives better deserves a medal of valor, and should not feel embarrassed about the scars. What other kids do they know that suffer such difficulties?

My younger brother has one son. My Mom says she could feel the bumps on my nephew's ribs when he was a baby, but she didn't say anything to anybody at the time. As each grandchild came along, she carried more guilt for passing on this abnormal affliction. By the time he was four years old, I noticed that he had that same curvature of the forearms. He has had surgeries to remove tumors from his arms and legs.

My younger sister, was born 1952. She had no obvious outward signs of MHE while growing up. Unlike the rest of us, she could bend herself into a pretzel shape. Even though she has the short stature, the widening of the top of the leg bones, called diaspheal aclasia, and a slightly deformed thumb, none of us comprehended that she also had MHE. It wasn't until she was a teenager, when our family took part in a medical research program, at the University of Buffalo, when it was discovered that she had well hidden smaller exostoses on her scapula and ribs as well as that thumb. My sister had two children, a girl, and a boy. Her daughter had an exostosis on her skull, located on the back of her head (occipital bone) My niece now has six children. My nephew has one daughter. Two of those children have signs of mild MHE.

There were no signs of the hereditary bone bumps on me at birth. At a very early age, though, I became aware of the unusual lumpy appearance of my mother's legs, and soon learned of my big brother's bumpy bones. By the time I was about nine years old, I was certain I had a couple of bumps, but no one in my family would believe me. It was commonly believed that girls didn't get them at all. Today, that is known to be a fallacy. Girls can have MHE, also. It is usually suggested that boys get worse cases of it than girls. But I suspect that is really an individual thing since, recently, I have gotten acquainted with many others through the MHE Coalition. Sometimes girls have more bone tumors than boys, and vice versa.

As I was growing up, I was very physically active in all kinds of sports. I loved participating in running, swimming, skating, baseball, field hockey and basketball. I became aware that my limitations were in anything requiring long distances, and stamina. I got lots of aches, and pains from ordinary play as well as sports activities. I thought it was normal. I thought that everyone had this kind of pain, so I never mentioned it, and just writhed with it sometimes. I thought I was a wimp when I couldn't keep up with other kids, wondering how they could smile right through it all. My strengths were in short sprint activities and anything that did not require a lot of group competition.

When I was thirteen, while skating with friends, I fell and injured an exostosis on my leg, close to my knee, causing me to be crippled for several weeks. It was at that time, in the emergency room, when my parents were finally able to acknowledge I had inherited the benign bone tumors, also. It was very hard for my mother to accept, and she spoiled me for the six weeks or so that it took for me to walk again.

After I married, I gave birth to three children, two girls and a boy. I thought that my first born had no signs of MHE until about age six, when I began to notice she walked a little knock-kneed. This is called Valgus Deformity. By the time she was about fourteen, she was scheduled to have surgery, but it was canceled at the last minute due to another medical condition we didn't know she had. Her surgery was postponed and she has decided to continue to live with the discomfort, and has never gone back to have any surgery for any of her cartilaginous tumors. Though, recently, she has begun to consider having surgery to see if it would relieve her of some of the biomechanical problems she deals with. I guess it is hard for her, remembering a childhood with a Mama who had lots of scary surgeries.

She has problems with Fibromyalgia and Osteo-Arthritis. I am aware the early onset arthritis is commonly related to MHE, as I have it too. But, I wonder if the Fibromyalgia could be directly related to MHE, also? So many members of my family have it. I understand that MHE researchers are presently looking into this possibility.

My daughter has had five children. One of her sons, inherited MHE, has had surgery upon his ankles, and the lower part of the two leg bones (Tibia and Fibula) where his bone tumors were lodged, preventing normal growth of his legs. Now a young adult, he works out regularly in a gym, participates in heavy duty sports, including wrestling competitions, which he has won, and works as an EMT. It appears that having surgery has helped him to be more active and flexible than those of us in the family who have not.

I have two granddaughters, through my first daughter. One was born with an obvious chondroma on her spine, and has developed others in different parts of her body over the years that did not appear to be there at birth. My other granddaughter did not seem to be affected. However, as the years have passed, it is now obvious that she has the valgus deformity of the ankles and knees. She has shorter stature and stockier build than her sister, plus she has exostoses in her wrist and shoulder. Both girls have some toe problems. Yes, it can affect fingers and toes. Though both have already had surgeries by the age of 9 they are active in dance classes. Did I tell you they are twins?

My other adult daughter is of very short stature. The ends of her bones have a characteristic wideness like my mother and sister. However, she has never been to a Orthopedic Tumor Specialist for a diagnostic work-up as having MHE, since distant family members raised her while I was ill. She has three daughters. I do not know if any of them have been diagnosed with MHE. Only time will tell. I hope not.

I knew that my son had MHE before he was two years old. I could feel the lumps in his wrists and on his ribs. By the time he was eight I took him to a specialist who did a complete set of head to toe x-rays, called a skeletal survey. This is usually the first diagnosic imaging that is done for MHE. Bone scans are not always appropriate for diagnosing exostoses because it can be mis-read as many "hot spots" and mis-interpreted as advanced cancer when it is not. So, if an x-ray shows an osteo chondroma, then a bone scan might be appropriate to determine locations of others. My son has chondromatoses all over his body, more than I have ever seen before in any family member. By the time he was 13, he was having many problems with his legs locking up on him. The muscles or tendons would "catch" on a tumor and he would not be able to unbend his legs. He had his first surgery that year to remove about five exostoses from one of his legs. And two years later, more were removed from the other leg. He had a third surgery, to remove one growth that grew back again, after previously being removed. Now, as an adult he has decided he does not want to bring any children into this world because they might also inherit MHE. This makes me sad, but I support him on his choices.

There is a form of genetic testing one can have called EXT 1 or EXT 2. In my family we have the EXT 2 form. If a person wants to have a child without MHE, she can have in vitro fertilization where the EXT has been removed. A specialized genetic clinic will need to be consulted for this. More information is available from the MHE Coalition.

I can't say that I have handled having this hereditary condition very well. However, I have adopted my son's philosophy about it. One day I overheard him say to someone who expressed sympathy to him about having it: "After a while, you get used to it."

Consider this analogy: It is like walking around with a new pair of shoes. At first, you think..."Oh, dratted shoes!" then, maybe they begin to irritate, and maybe you get annoyed. Then, perhaps angry, or feeling sorry for yourself, because it is uncomfortable. Then, it REALLY begins to hurt, becomes blistered, maybe gets raw, bleeding, infected, and you suffer, and hate it. Maybe you break down and cry. But, since it's permanent and you can't make it go entirely away, you begin to grow a callous around it. The callous grows big and tough, and makes it much more tolerable. You adapt your lifestyle around it. You quietly accommodate yourself to your needs, and get along with it the best you can. And, finally, for the most part, you don't even think about it anymore. It is then you realize, like my son says, "You get used to it".

I think that we all adapt to whatever disabilities we may have. Some adapt easier than others. Some fight it every step of the way. I have learned that I have two choices. I can be miserable about it, or I can LIVE LIFE TO THE FULLEST EXTENT OF MY BEING, (even with the difficulties).

I have already mentioned that when I was a kid I was active and sporty, a "Tomboy," they used to call me. I played baseball, rode bicycle, ran races, ice-skated, basketball, field hockey, you name it, I tried it! I married right out of high school and had my two daughters. Then, noticed one of the tumors growing bigger, and had the surgery to remove it. At that time it was thought to be benign, so no surrounding tissue was removed with the tumor. After the second surgery, where a portion of my pelvis (Internal Hemi-Pelvectomy) was cut away, I had to stop participating in my favorite activities. It broke my heart, and in some ways broke my spirit.

Then, some people encouraged me, or goaded me to participate in these things after I knew it was no longer safe for me to do so. The doctor had warned me of the dangers. I missed those activities so much, and was easily convinced to try them again. But, I have had to pay a high price of suffering additional unecessary injury to my body. I have lost those well-meaning friends who were disappointed in me that I didn't "overcome" and use "mind over matter". When a portion of your body has been amputated no matter how much you visualize or pray or wish, you cannot grow new bone to replace that which has been surgically removed.

I have learned to respect the messages my body gives me. After jogging and having the right leg pop out of the hip joint, and another time I fell and ripped open the inner part of my groin herniating my bladder, I no longer give in to peer-pressure. This resulted in an extended hospital stay. Another time I was just walking on a slippery sidewalk after a rain. Because some of the pelvic floor muscles have been removed, as well as the adductor muscle, I had no way of preventing my leg from violently sliding out from under me. I did a complete split, which tore the hamstring muscle out from the upper femur, (thigh bone). I was crippled for months. Unfortunately, at that time, no surgery was performed to repair the damage, so now, my right leg is very unstable. It could happen again with any careless step. Now, the only really safe exercise I can do is walk and swim. Thank heavens, I still love to swim!

I hate being hampered by the exostoses. The average person looks at me and cannot tell that there is much of anything disabling me. Sometimes others may judge me as lazy, wimpy or whatever. I have Osteoarthritis problems, but have learned to stay limber and in shape as much as I can. There have been times that I have been crippled enough to require a wheelchair when things got bad. I could have stayed there, in ignorance, and let people wait on me. But, I have learned that with the proper physical therapy, there is always room for improvement no matter how debilitated one is. The ability to stay upright and walking is my daily priority.
ABOUT THE CHONDROSARCOMA

I hate having surgery! And I hate submitting myself to medical treatment. I often felt like I was giving up part of my soul to be in the hospital, and under the care of others. I have since overcome my attitude and learned to understand that I am only as helpless as I allow myself to feel, even as a patient unable to turn myself in a bed. I truly cannot criticize medical caretakers too much, as I would not be here today, if I had not gone through with the necessary medical treatment that was available to me. And I know for sure that I would never have the patience and stamina to be a caretaker of patients in a hospital setting. Some of the Medical Professionals with whom I have come into contact have been the epitome of Healing Angels in disguise, human beings with open hearts.(Most of my Orthopedic Oncologists, for example, many wonderful committed Nursing staff and Physical Therapists, too). People who work in the medical community usually do so with a great commitment for really wanting to succeed in helping people to be healthy and well. Sometimes, I have had to remember they are human beings also, and need to be understood as not perfect and all knowing of the medical answers.Too often, we have high expectations and are quite judgmental of those in whose hands we put our care. We need to share the responsibility for our health and that keeps us for faulting those with whom we share the job. When necessary, we have the ability to get another opinion.

The first Chondrosarcoma tumor was surgically removed when I was about the age of twenty-two. This is the time when our bodies go through their very last little growth spurt, when the two separate bones of the pubis join together, forming the pubis symphysis. The orthopedic surgeon I saw was in a military hospital far from any big city. He operated on me at the time but apparently didn't know too much about it. He didn't think it was cancer anything but a chondroma and just sawed it off like they would any other benign cartilage tumor. Apparently he didn't know that he should have taken out a lot of surrounding bone, and tissue to prevent recurrence. But, then he certainly didn't expect a recurrence. I am pretty sure that the pathology department didn't have the ability to recognize that it was chondrosarcoma. I have learned that sometimes it is very difficult to diagnose. Since the tumor was not benign, after all, it began showing obvious growth within six months of the surgery, but it was my understanding that it was just bony scar tissue. Unfortunately, the tumor came back very aggressively, and within two years I had a huge amount of cancer growing inside my pelvis, (well hidden,) and a chunk of it growing down the inside of my thigh. This was probably a good thing, otherwise I probably would not have noticed the growth and sought help until it was too late.

In embarking upon my second surgery, the Orthopedic Oncologist at University of Buffalo, Dr. Eugene Mindell, was very cautious about giving me a long-term prognosis. At the time, a five-year survival rate was rare for the size and location of my tumor. I guess I was one of the lucky ones, or maybe not. There have been plenty of times I have wondered why I am still alive. And just as often wished I was dead, (as I mentioned before, I had some emotional maturing to go through). I wondered why I had to have so many recurrences, so many surgeries, so much pain and suffering. I wondered why there was no chemo or radiation to help me. I wondered if it would have been better for me to have had my whole leg removed above the hip joint, a complete hemipelvectomy, as originally suggested.

Usually, doctors (Bone Tumor Specialists) would have removed as much pelvis (including the leg) as possible, during that time period. Internal hemipelvectomy is what they call it when they cut out part of the pelvis. In my case, the huge tumor was removed from its attachments to my internal organs, the bladder, birth canal and rectum along with parts of these organs. They were all surgically rebuilt with what was left of them. All the connected muscles in the area were removed also, along with associated lymph glands. Each year the tumor grew back and each year I discovered the new growth, and returned to the doctor to submit myself to more surgical torture. Each year more of the pelvic bone was sawed away. Yes, they do use a tool exactly like a saw. And, sometimes a hammer and chisel, too. I kid you not!

But, I had a compassionate doctor who cared what a 23-year-old girl would be living with. So, he saved my leg, cutting out as much pelvis as he could. This is called Limb Salvage. Unfortunately, the tumor kept on growing back resulting in my many surgeries over the years. I have lived in different parts of the country and have had different Musculoskeletal Tumor Specialists or Orthopedic Oncologists take care of me. It is always best to be treated for Bone Tumors at University Medical Centers because they are so very rare. A regular physician probably never sees one, and seldom diagnoses one. An Orthopedic surgeon is more likely to diagnose, but would refer to an Orthopedic Oncologist. One who is associated with a large University Medical center is going to have the most experience treating bone tumors.

The last recurrence was in 1980. It was considered fortunate that I had a "low grade" cancer. What did they mean by that? The numbering of a cancer's "grade" or "staging" is very important. It is a good indicator of how much one should be concerned, and follow up with getting medical attention. With Chondrosarcoma, they give it a number from 1 to 4. A Number 1 pathology is very slow growing, Number 4 is very aggressive.

Depending on the number, it can take up to twenty years to metastasize to your lungs or elsewhere. That means, as late as twenty years after the last tumor was removed, it could seed itself somewhere else in my body and spread there. So, full body Bone Scans, Cat Scans, or MRI's should be done on a regular basis.

Since I have had so many surgeries, about a third of my pelvis has been removed along with adjacent muscle, lymph nodes, as well as muscle in my thigh etc. Because of this I have been on Social Security Disability since 1967.The damage is permanent. I have survived the onslaught of cancer, the pain and torture of modern medical practice. It is still quite barbaric! Doesn't anyone realize that? I have survived all the dire predictions of my demise.

What do doctors know? Nowadays, because of people like me, who have long-term survival, the prognosis is not quite so guarded for this type of bone cancer. My biggest regret is that they never put any sort of prosthesis inside me. You know, perhaps a steel beam connected to one leg socket stretched across to the other to give my body a chance for balance and strength. It is my understanding that if prosthesis is placed too close to the surgery for chondrosarcoma, it can be problematical in case there is recurrence. Also, infections can be a serious. Medical Science has come a long way and now limb sparing pelvic surgeries with prostheses are very successful.

Why is a prosthesis so important to me? You may ask. Think of the Houston Astro-Dome. Because of the way it is built, each piece is dependent on the others for its strength and endurance. Weaken one part of the infrastructure and eventually the whole thing crumbles. If you removed part of the Astro-Dome (without supporting it) the wind could just blow it down, Because of this, what is left of my pelvis has been overworked tremendously. It just sort of swings loosely like a gate. Without the support that was intended to hold it together, it has weakened and been worn away. The sacroiliac joints have degenerated to the point that there is no more cartilage left to cushion them, and now bone rubs against bone. The pain never leaves. It is my constant companion. It varies, and I have learned to deal with it in many ways. Yes, there are times when I have to walk with a cane, or a walker. There have been times when I have to use the wheel chair, and times when being bedridden is the only option. It changes. I never know what tomorrow might bring.

I used to feel like Frankenstein's bride with all those ugly red railroad tracks crisscrossing my lower body, from naval to knee. But, I have learned to love my body the way a mother loves her ugly child, tenderly and compassionately, but sensitive of the unkind judgment of the ignorant. There was a researcher in who looked for people like me for a donation of a tube of blood for study. They are now able to determine if you have a Multiple Hereditary Exostoses. All the living generations of my family have been a part of this project. You can learn more about the results from the MHE Coalition.

And that's the short form of my story. There is so much more to tell, but now for some information regarding the genetic connection:

Please be aware and assured that just because one has multiple exostoses (some are not inherited, by the way) one does NOT inherit chondrosarcoma. Though, a little higher percentage of Chondrosarcoma will appear in those with MHE than with the normal population.

It can be very confusing to research Chondrosarcoma for the layperson. There are so many kinds of Bone Tumors, Bone Cancers and Sarcomas. One can get confused sorting out the facts. My doctors have taught me a great deal. I have attended pre-med courses in college. I have learned to do appropriate research. ( see: www.pubmed.com ) I hope to help others from having to spend time in their lives trying to sort out the facts from the fiction in learning about Chondrosarcoma, when they need to be facing the diagnosis, treatment options, and recovery.
MORE ABOUT BONES AND TUMORS IN GENERAL

The following information is for a layperson's understanding. It is important to check out the facts for yourself.

What are bones made of and how do they function? Mature bones are made up of three types of tissue: compact tissue (the hard outer portion of most bones); cancellous tissue (spongy tissue inside the bones that contains bone marrow, which makes blood cells); and subchondral tissue (smooth bone tissue of the joints). A layer of cartilage covers subchondral tissue to cushion the movement of joints. Bones support and protect internal organs, act as levers and braces for muscles to produce movement, and produce and store blood cells in the bone marrow.

Bone tumors can be divided into four categories - benign primary, (such as an exostoses or enchondroma) malignant, (such as a chondrosarcoma) metastatic, (such as when cancer spreads to the bones) and lesions that simulate bone tumors, (such as myeloma or lymphoma). Sarcomas are malignant tumors of connective tissue origin. Soft tissue tumors are benign or malignant and rarely metastasize to bone.

Most (but not all) patients with bone tumors present with pain near where the tumor is located. The pain is typically described as dull, deep, aching pain, sometimes worse at night interrupting sleep. Some people say they do not experience pain with their bone tumors. It can depend on whether or not a tumor is pressing up against a nerve, tendon, muscle, or other body tissue.

Sometimes the doctor will order a complete set of x-rays called a skeletal (bone) survey, if there is reason to suspect Multiple Hereditary Exostoses. Bone Scans, following up with CAT scans or MRI. are other tests useful for determining maligancy, and depending on many factors which should be discussed with the doctors. Sometimes insurance coverage can make a difference as to which tests will be ordered first.

There is a difference between Bone Tumors and Cartilage Tumors. Cartilage forming tumors include those called: enchondroma, osteochondroma, chondromyxoid fibroma, chondroblastoma, and chondrosarcoma. Bone forming tumors include osteoid osteoma, osteoblastoma, osteochondroma, osteosarcoma, and metastases.

X-rays and scans are not entirely enough to determine what a particular bone growth might be. Biopsy is necessary to verify diagnosis. It is helpful for the pathologist, who looks in the microscope at the tumor specimen, and the surgeon to have a fairly good idea of what to expect before the time of the biopsy. That is why, sometimes, it takes more than one test and one visit to the doctor to decide how to proceed with each individual patient's situation. It is considered to be imperative that the actual biopsy be done at the center where the treatment is to be given. Second opinions are also helpful.

According to many sources: "The treatment of musculoskeletal tumors requires a team approach, including an orthopaedic tumor surgeon, pathologist, medical or pediatric oncologist, (occasionally) radiation therapist, social worker, and physical therapist. Once a tumor is suspected, the patient should be immediately referred to a Musculoskeletal Oncologist or Orthopedic Oncologist for evaluation." The best place to get treatment for bone cancers is at a University Medical Center with a Sarcoma Clinic.

A musculoskeletal oncologist is the kind of doctor who makes the decisions about proper staging, biopsy, and treatment as appropriate. It is important for patients to invest time and energy in finding the right kind of doctor. They should be referred only to orthopedists who specialize in bone tumor management.

If malignancy is located in the spine, ribcage, skull or throat, a specialist in these areas who has had chondrosarcoma experience would be required, and best referred by those same Orthopedic Oncologists who one finds in the Sarcoma Clinic.

MHE description

MULTIPLE HEREDITARY EXOSTOSES DESCRIPTION

Multiple Hereditary Exostoses (MHE) is a rare inherited disorder of benign bony tumors also known as Osteochondromatoses, or Multiple Cartilaginous Exostoses or Diaphyseal Dysplasias, as well as other names. MHE is a condition that is passed on by the genes of the affected parent. Rarely it can begin in a person whose family members do not have it, but it is then possible to be passed on to descendants of that affected person. It is called an autosomal dominant disorder, which means that if one parent has the condition, chances are fifty percent that any child born to them could also inherit MHE. Recently, chromosomes 8, 11, and 19 have all been shown to be locations where the genetic information for MHE is located. People who have MHE grow bumps (benign cartilage bone tumors) on their bones, which can vary in size, location, and number.

An exostosis is a bone growth that is abnormal, growing differently from the appropriate underlying architecture of the bone. There is no consistency to the shapes in which they can grow. Some are like mushrooms, some like hooks; some are oddly shaped flattened lumps. They can be rounded or sharp, and continue to grow while a child is growing up to as late as age twenty-two. These "abnormal growths" are benign. Sometimes doctors refer to exostoses as "tumors" which is a general term meaning abnormal growth. It is important to remember that not all "tumors" are cancer. Most tumors, like the exotoses of MHE, are benign.

MHE can be troublesome. The exostoses locate near the growth centers of the bone, near the ends of the bones, and nearby the joints. This can make the growth center grow poorly. This makes a lot of people with MHE somewhat shorter than average or have bowed arms or legs. Often, the forearm will bow out toward the little finger, or the legs can become knock-kneed (valgus deformity). Sometimes folks with MHE have less range of motion, especially in the elbows and hips, usually because their exostoses block some of their motion.

While children are growing, exostoses can be sensitive when bumped. Kids often develop exostoses on the inside of their knees and these can hit together when they run. Sometimes exostoses grow near nerves or tendons and press on them, causing pain. In these cases, they may need to be surgically removed so they won't damage the structure laying over them. Once removed, exostoses can reoccur (about 20 - 50% of the time), but may not grow back to a size large enough to be symptomatic. If an exostosis causes a growth abnormality, like bowing, sometimes just removing the exostoses at an early age will allow the bone to straighten itself out as the child grows. Some bowing is so severe that not only must the exostoses be removed, but also the bone must be straightened. This can be done by either cutting the bone, straightening it and then holding it in place while it heals or if the child is still growing by changing the rate of growth on one side of the growth plate.

Some people with MHE never seek any treatment. They learn to compensate for their deformity or decreased range of motion so they function somewhat normally or are unaware of their deficits. When deformity does occur, it often happens so slowly that the patient can compensate for it well, while others may have such noticeable symptoms as to require surgery to help them. A specialist in orthopedic bone tumors would be required to do this.

The most frightening complication of MHE is also one of the most uncommon. Rarely, a benign exostoses can become a malignant tumor called chondrosarcoma. This happens almost always after adulthood when skeletal growth has ceased. Usually, patients who develop chondrosarcoma are in their 20's to 50's. If a person with MHE notices that an exostoses is getting bigger or painful after they reached adulthood, they should get to their doctor! Growth and pain are two important warning signs that a benign tumor has become malignant. If an exostoses does become malignant and turn into a Chondrosarcoma then it must be removed as soon as possible. Chondrosarcoma is very rare, but MHE families must be aware of this possibility. The survival rate for Chondrosarcoma is favorable, if treated early. Fear of the word, "Cancer" is usually the biggest factor that people with chondrosarcoma have to face.
MHE and CHONDROSARCOMA RESOURCES
 
MHE COALITION
This is the ultimate source for Hereditary Multiple Exostoses information and support.
MHE and Me Handbook, a Guide for Family, Friends, Teachers and Classmates
a Guide for Family, Friends, Teachers and Classmates. What is MHE? How are children affected? How can you help others to understand?

Adult Bone Cancer Survivor's
We are a group of adults from all over the world who have been diagnosed with bone sarcoma, a type of cancerous tumor that invades bone.
Chondrosarcoma Questions and Answers.
A Patient's Guide to Chondrosarcoma written in plain language.
Survived 2B Alive
Chondrosarcoma and other Sarcoma Patient Stories & Information
Chondrosarcoma Support Group
A support group forum for chondrosarcoma patients, their loved ones, caregivers, family, friends and medical staff who are interested in chondrosarcoma. No one need go through this alone.
Learn About Musculo-skeletal Conditions
contains info including exostoses, endocohondromas, limb reduction, sarcoma, etc
Kristen's pelvis chondrosarcoma journey
Ben Ling Chondrosarcoma Research Fund
Donate to Chondrosarcoma Research here.
University of Washington, Radiology Dept.
Approaches To Differential Diagnosis In Musculoskeletal Imaging, Skeletal Dysplasias by Michael L. Richardson, M.D. Written from a natural human perspective.
Ortho Dept, Dupont Hospital For Children, Wilmington, DE
OSTEOCHONDROMA (Multiple Exostosis) A clinical case presentation. Easier to read than some
MHE, Multiple Hereditary Exostoses, or Osteochondromatosis
This is the story of Bill and his search for others with MHE. Bill has a link for a group site for people interested in MHE.
MHE and Me, a Support Group for Kids
You don't have to be a kid to find this site useful!!
Touch of Hope Carly Laverty
Long term survivor, Carly and her history of Mesenchymal Chondrosarcoma
Eric's Sarcoma Fight
Eric has Sarcoma in the Jaw/mandible. This is his journal web log.
Hemipelvectomy and Disarticulation
web site specifically dedicated to the high pelvic levels of amputation, HemiPelvectomy and Hip-Disarticulation.
My Brother's MHE Story
            This is the way my brother experienced his MHE. 
Lucent Lesions of Bone
Approaches To Differential Diagnosis In Musculoskeletal Imaging
The Liddy Shriver Sarcoma Initiative
Chondrosarcoma of Bone: Article by R. Lor Randall, MD, FACS
Adult Bone Cancer Survivors Learn about Chondrosarcoma
Read about other Survivor's of chondrosarcoma
Musculoskeletal Tumor Society Doctor's List
Find the most qualified doctor to treat bone tumors here.
Patient Advocate Foundation
safeguard patients through effective mediation assuring access to care, maintenance of employment and preservation of their financial stability.
Hereditary Multiple Exostoses Support Group
Very informative site. Located in Britain. Check it out.
Bare Bone Radiology
An explanation radiology exams, x-rays, MRI, CatScans etc.
Genotype-phenotype correlation in hereditary multiple exostoses
A genetic description
Livestrong Patient Survivor's Stories
Elizabeth's interview in print and video
Osteonecrosis of left hip
Multiple hereditary exostosis syndrome involving right proximal femur
Gene Expression of Human Sarcomas
A, publicly available, searchable database housing the data from the gene expression profiles of these tumors
Choosing a Doctor
how to deal with your HMO choosing your Primary Care Physician
Patient's Bill of Rights
Did you know you have Rights??
Experimental Therapeutics, Preclinical Pharmacology
Antiangiogenesis Treatment Combined with Chemotherapy Produces Chondrosarcoma Necrosis
Accessing Your Medical Records
Keeping track of your medical records, even keeping your own records, is an important part of dealing with any serious medical problem. But especially with MHE and Chondrosarcoma
What is Sarcoma
easy to understand discussion which includes chondrosarcoma
My Dog has Chondrosarcoma
Mike's own description of his Chondrosarcoma with help from his beloved person
Doug Ulman's Story
A defender on Brown's soccer team, Doug Ulman goes fought his own cancer and help others in similar situations
Risk Factors for Cancer
Air and Water Pollution
National Cancer Institute
Post Traumatic Stress Disorder and risk for cancer
excellent info for newly diagnosed cancer patients
Treatments, Attitudes, Knowledge for self help
Mesenchmal Chondrosarcoma
One young boy's successful journey through this very rare cancer
Self Care Cancer Pain Management Log
You can print out this log to keep a record of how well your medication is working to control your pain.
Pathology of bone and joints
National Patient Travel
provides information about all forms of charitable, long-distance medical air transportation etc.

Cornucopia
Disability information