Chondrosarcoma
Encyclopedia
Chondrosarcoma is a cancer
composed of cells derived from transformed cells that produce cartilage
. Chondrosarcoma is a member of a category of "soft tissue" malignancies known as sarcoma
s. About 30% of skeletal system cancers are chondrosarcomas. While the disease can affect people (or animals) of any age, unlike most other forms of skeletal system cancer, it is more common among older people than among children, and more often affects the axial skeleton
than the appendicular skeleton
.
).
Grade 1 chondrosarcoma grows relatively slowly, has cells whose histological appearance is quite similar to cells of normal cartilage, and have much less aggressive invasive and metastatic properties. Grades 2 and 3 are increasingly faster-growing cancers, with more varied and abnormal-looking cells, and are much more likely to infiltrate surrounding tissues, lymph nodes, and organs. Some, but not all, authorities and medical facilities assign a "Grade 4" to the most anaplastic, undifferentiated cartilage-derived tumors.
The most common sites for chondrosarcoma to grow are the pelvis and shoulder, along with the superior metaphysial
and diaphysial
regions of the arms and legs. However, chondrosarcoma may occur in any bone, and are sometimes found in the skull, particularly at its base.
ICD-O codes provide a more precise classification of chondrosarcoma. These "subtypes" are derived from, and reflect, both (a) the topographical location of the tumor, (b) the histological characteristics of the cancerous cartilage cells, and (c) the makeup of the surrounding matrix material associated with the tumor:
(MRI) - are often used to make a presumptive diagnosis
of chondrosarcoma. However, a definitive diagnosis depends on the identification of malignant cancer cells producing cartilage in a biopsy
specimen that has been examined by a pathologist. In a few cases, usually of highly anaplastic tumors, immunohistochemistry
(IHC)is required.
There are no blood tests currently available to enable an oncologist to render a diagnosis of chondrosarcoma. The most characteristic imaging findings are usually obtained with CT.
Nearly all chondrosarcoma patients appear to be in good health. Often, patients are not aware of the growing tumor until there is a noticeable lump or pain. Earlier diagnosis is generally accidental, when a patient undergoes testing for another problem and physicians discover the cancer. Occasionally the first symptom will be a broken bone at the cancerous site. Any broken bone that occurs from mild trauma warrants further investigation, although there are many conditions that can lead to weak bones, and this form of cancer is not a common cause of such breaks.
or osteochondroma
. A small minority of secondary chondrosarcomas occur in patients with Maffucci syndrome and Ollier disease.
Surgery is the main form of treatment for chondrosarcoma. Musculoskeletal tumor specialists or orthopedic oncologists are usually chosen to treat chondrosarcoma, unless it is located in the skull, spine, or chest cavity, in which case, a neurosurgeon or thoracic surgeon experienced with sarcomas is chosen. Often, a limb-sparing operation can be performed, however in some cases amputation is unavoidable. Amputation of the arm
, leg
, jaw
, or half of the pelvis
(called a hemipelvectomy
) may be necessary in some cases.
There are two kinds of hemipelvectomy (internal and external)
Amputation at the hip is called hip disarticulation and amputees who have had this amputation are also called hip disartics.
Chemotherapy or traditional radiotherapy are not very effective for most chondrosarcomas, although proton therapy
is showing promise with local tumor control at over 80%.
Complete surgical ablation is the most effective treatment, but sometimes this is difficult. Proton therapy
Radiation can be useful in awkward locations to make surgery more effective.
Recent studies have shown that induction of apoptosis
in high-grade chondrosarcoma, both directly and by enhancement of response to chemotherapy
and radiation
, is a valid therapeutic strategy.
Tumors may recur in the future. Follow up scans are extremely important for chondrosarcoma to make sure there has been no recurrence or metastasis, which usually occurs in the lungs.
Cancer
Cancer , known medically as a malignant neoplasm, is a large group of different diseases, all involving unregulated cell growth. In cancer, cells divide and grow uncontrollably, forming malignant tumors, and invade nearby parts of the body. The cancer may also spread to more distant parts of the...
composed of cells derived from transformed cells that produce cartilage
Cartilage
Cartilage is a flexible connective tissue found in many areas in the bodies of humans and other animals, including the joints between bones, the rib cage, the ear, the nose, the elbow, the knee, the ankle, the bronchial tubes and the intervertebral discs...
. Chondrosarcoma is a member of a category of "soft tissue" malignancies known as sarcoma
Sarcoma
A sarcoma is a cancer that arises from transformed cells in one of a number of tissues that develop from embryonic mesoderm. Thus, sarcomas include tumors of bone, cartilage, fat, muscle, vascular, and hematopoietic tissues...
s. About 30% of skeletal system cancers are chondrosarcomas. While the disease can affect people (or animals) of any age, unlike most other forms of skeletal system cancer, it is more common among older people than among children, and more often affects the axial skeleton
Axial skeleton
The axial skeleton consists of the 80 bones along the central axis of the human body. It is composed of six parts; the human skull, the ossicles of the middle ear, the hyoid bone of the throat, the rib cage, sternum and the vertebral column...
than the appendicular skeleton
Appendicular skeleton
The Appendicular skeleton is composed of 126 bones in the human body. The word appendicular is the adjective of the noun appendage, which itself means a part that is joined to something larger...
.
Classification and Grading
Physicians grade chondrosarcoma using several criteria, but particularly on how abnormal the cancerous cells appear under the microscope, and the growth rate of the tumors themselves, both of which are directly linked to the propensity of the cancer to invade locally, and to spread widely to distant organs and sites in the body (called metastasisMetastasis
Metastasis, or metastatic disease , is the spread of a disease from one organ or part to another non-adjacent organ or part. It was previously thought that only malignant tumor cells and infections have the capacity to metastasize; however, this is being reconsidered due to new research...
).
Grade 1 chondrosarcoma grows relatively slowly, has cells whose histological appearance is quite similar to cells of normal cartilage, and have much less aggressive invasive and metastatic properties. Grades 2 and 3 are increasingly faster-growing cancers, with more varied and abnormal-looking cells, and are much more likely to infiltrate surrounding tissues, lymph nodes, and organs. Some, but not all, authorities and medical facilities assign a "Grade 4" to the most anaplastic, undifferentiated cartilage-derived tumors.
The most common sites for chondrosarcoma to grow are the pelvis and shoulder, along with the superior metaphysial
Metaphysis
The metaphysis is the wider portion of a long bone adjacent to the epiphyseal plate. It is this part of the bone that grows during childhood; as it grows, it ossifies near the diaphysis and the epiphyses...
and diaphysial
Diaphysis
The diaphysis is the main or mid section of a long bone. It is made up of cortical bone and usually contains bone marrow and adipose tissue ....
regions of the arms and legs. However, chondrosarcoma may occur in any bone, and are sometimes found in the skull, particularly at its base.
ICD-O codes provide a more precise classification of chondrosarcoma. These "subtypes" are derived from, and reflect, both (a) the topographical location of the tumor, (b) the histological characteristics of the cancerous cartilage cells, and (c) the makeup of the surrounding matrix material associated with the tumor:
- 9220 = Chondrosarcoma NOS ("Not Otherwise Specified")
- 9221 = Juxtacortical chondrosarcoma
- 9231 = Myxoid chondrosarcoma
- 9240 = Mesenchymal chondrosarcomaMesenchymal chondrosarcomaMesenchymal chondrosarcoma is a form of malignant chondrosarcoma. Unlike most chondrosarcomas, mesenchymal chondrosarcoma grows rapidly, tends to spread, and occurs more often in children and young adults than in older adults....
- 9242 = Clear cell chondrosarcoma
- 9243 = Dedifferentiated chondrosarcoma
Diagnosis
Imaging studies - including radiographs ("x-rays"), computerized tomography (CT), and magnetic resonance imagingMagnetic resonance imaging
Magnetic resonance imaging , nuclear magnetic resonance imaging , or magnetic resonance tomography is a medical imaging technique used in radiology to visualize detailed internal structures...
(MRI) - are often used to make a presumptive diagnosis
Diagnosis
Diagnosis is the identification of the nature and cause of anything. Diagnosis is used in many different disciplines with variations in the use of logics, analytics, and experience to determine the cause and effect relationships...
of chondrosarcoma. However, a definitive diagnosis depends on the identification of malignant cancer cells producing cartilage in a biopsy
Biopsy
A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...
specimen that has been examined by a pathologist. In a few cases, usually of highly anaplastic tumors, immunohistochemistry
Immunohistochemistry
Immunohistochemistry or IHC refers to the process of detecting antigens in cells of a tissue section by exploiting the principle of antibodies binding specifically to antigens in biological tissues. IHC takes its name from the roots "immuno," in reference to antibodies used in the procedure, and...
(IHC)is required.
There are no blood tests currently available to enable an oncologist to render a diagnosis of chondrosarcoma. The most characteristic imaging findings are usually obtained with CT.
Nearly all chondrosarcoma patients appear to be in good health. Often, patients are not aware of the growing tumor until there is a noticeable lump or pain. Earlier diagnosis is generally accidental, when a patient undergoes testing for another problem and physicians discover the cancer. Occasionally the first symptom will be a broken bone at the cancerous site. Any broken bone that occurs from mild trauma warrants further investigation, although there are many conditions that can lead to weak bones, and this form of cancer is not a common cause of such breaks.
Causes
The cause is unknown. Patients may have a history of enchondromaEnchondroma
An Enchondroma is a cartilage cyst found in the bone marrow. Typically, enchondroma is discovered on a X-ray scan. Enchondromas have a characteristic appearance on Magnetic Resonance Imaging as well. They have also been reported to cause increased uptake on PET...
or osteochondroma
Osteochondroma
Osteochondroma is a type of benign tumor that consists of cartilage and bone. It is a benign capped-cartilage outgrowth, connected to bone by a stalk.It is the most frequently observed neoplasm of the skeleton....
. A small minority of secondary chondrosarcomas occur in patients with Maffucci syndrome and Ollier disease.
Treatment
Treatment depends on the location of the disease and the aggressiveness of the tumors. Because chondrosarcomas are rare, they are treated at specialist hospitals with Sarcoma Centers.Surgery is the main form of treatment for chondrosarcoma. Musculoskeletal tumor specialists or orthopedic oncologists are usually chosen to treat chondrosarcoma, unless it is located in the skull, spine, or chest cavity, in which case, a neurosurgeon or thoracic surgeon experienced with sarcomas is chosen. Often, a limb-sparing operation can be performed, however in some cases amputation is unavoidable. Amputation of the arm
Arm
In human anatomy, the arm is the part of the upper limb between the shoulder and the elbow joints. In other animals, the term arm can also be used for analogous structures, such as one of the paired forelimbs of a four-legged animal or the arms of cephalopods...
, leg
Leg
Łęg may refer to the following places in Poland:*A former name for the town of Ełk *Part of the Czyżyny district of Kraków*Łęg, Pleszew County in Greater Poland Voivodeship...
, jaw
Jaw
The jaw is any opposable articulated structure at the entrance of the mouth, typically used for grasping and manipulating food. The term jaws is also broadly applied to the whole of the structures constituting the vault of the mouth and serving to open and close it and is part of the body plan of...
, or half of the pelvis
Pelvis
In human anatomy, the pelvis is the lower part of the trunk, between the abdomen and the lower limbs .The pelvis includes several structures:...
(called a hemipelvectomy
Hemipelvectomy
A hemipelvectomy is a high level pelvic amputation. Along with hip disarticulations, hemipelvectomies are the rarest of lower extremity amputations. In some cases, an internal hemipelvectomy can be performed, which is a limb-sparing procedure. A complete hemipelvectomy, however, is the...
) may be necessary in some cases.
There are two kinds of hemipelvectomy (internal and external)
- External hemipelvectomy - is removal of that half of the pelvis with the amputation of the leg. It is also called the hindquarter amputation.
- Internal hemipelvectomy - is removal of that half of the pelvis, but the leg is left intact.
Amputation at the hip is called hip disarticulation and amputees who have had this amputation are also called hip disartics.
Chemotherapy or traditional radiotherapy are not very effective for most chondrosarcomas, although proton therapy
Proton therapy
Proton therapy is a type of particle therapy which uses a beam of protons to irradiate diseased tissue, most often in the treatment of cancer. The chief advantage of proton therapy is the ability to more precisely localize the radiation dosage when compared with other types of external beam...
is showing promise with local tumor control at over 80%.
Complete surgical ablation is the most effective treatment, but sometimes this is difficult. Proton therapy
Proton therapy
Proton therapy is a type of particle therapy which uses a beam of protons to irradiate diseased tissue, most often in the treatment of cancer. The chief advantage of proton therapy is the ability to more precisely localize the radiation dosage when compared with other types of external beam...
Radiation can be useful in awkward locations to make surgery more effective.
Recent studies have shown that induction of apoptosis
Apoptosis
Apoptosis is the process of programmed cell death that may occur in multicellular organisms. Biochemical events lead to characteristic cell changes and death. These changes include blebbing, cell shrinkage, nuclear fragmentation, chromatin condensation, and chromosomal DNA fragmentation...
in high-grade chondrosarcoma, both directly and by enhancement of response to chemotherapy
Chemotherapy
Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....
and radiation
Radiation
In physics, radiation is a process in which energetic particles or energetic waves travel through a medium or space. There are two distinct types of radiation; ionizing and non-ionizing...
, is a valid therapeutic strategy.
Prognosis
Prognosis depends on how early the cancer is discovered and treated. For the least aggressive grade, about 90% of patients survive more than five years after diagnosis. People usually have a good survival rate at the low grade volume of cancer. For the most aggressive grade, more than a quarter of patients live more than five years.Tumors may recur in the future. Follow up scans are extremely important for chondrosarcoma to make sure there has been no recurrence or metastasis, which usually occurs in the lungs.
External links
- Chondrosarcoma of Bone
- Epidemiology of Bone Cancer: An Overview
- CancerBACUP Chondrosarcoma Info Page
- Bone Tumor.org Fact Page on Chondrosarcoma
- What is Sarcoma from the Sarcoma Alliance
- http://www.winnipegfreepress.com/local/emmiracle-emmom-103194769.html Miracle Mom survives Chondrosarcoma