Chondrosarcoma is a
bone sarcoma, a primary
cancer
Cancer is a group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body. These contrast with benign tumors, which do not spread. Possible signs and symptoms include a lump, abnormal bl ...
composed of cells derived from transformed cells that produce
cartilage
Cartilage is a resilient and smooth type of connective tissue. In tetrapods, it covers and protects the ends of long bones at the joints as articular cartilage, and is a structural component of many body parts including the rib cage, the neck a ...
.
A chondrosarcoma is a member of a category of tumors of bone and soft tissue known as
sarcoma
A sarcoma is a malignant tumor, a type of cancer that arises from transformed cells of mesenchymal (connective tissue) origin. Connective tissue is a broad term that includes bone, cartilage, fat, vascular, or hematopoietic tissues, and sarcoma ...
s. About 30% of bone sarcomas are chondrosarcomas. It is resistant to
chemotherapy
Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs ( chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemother ...
and
radiotherapy
Radiation therapy or radiotherapy, often abbreviated RT, RTx, or XRT, is a therapy using ionizing radiation, generally provided as part of cancer treatment to control or kill malignant cells and normally delivered by a linear accelerator. Rad ...
. Unlike other primary bone sarcomas that mainly affect children and adolescents, a chondrosarcoma can present at any age. It more often affects the
axial skeleton
The axial skeleton is the part of the skeleton that consists of the bones of the head and trunk of a vertebrate. In the human skeleton, it consists of 80 bones and is composed of six parts; the skull (22 bones), also the ossicles of the midd ...
than the
appendicular skeleton
The appendicular skeleton is the portion of the skeleton of vertebrates consisting of the bones that support the appendages. There are 126 bones. The appendicular skeleton includes the skeletal elements within the limbs, as well as supporting sho ...
.
Types
Symptoms and signs
* Back or thigh pain
*
Sciatica
Sciatica is pain going down the leg from the lower back. This pain may go down the back, outside, or front of the leg. Onset is often sudden following activities like heavy lifting, though gradual onset may also occur. The pain is often described ...
* Bladder Symptoms
* Unilateral
edema
Edema, also spelled oedema, and also known as fluid retention, dropsy, hydropsy and swelling, is the build-up of fluid in the body's tissue. Most commonly, the legs or arms are affected. Symptoms may include skin which feels tight, the area ma ...
Causes
The cause is unknown. There may be a history of
enchondroma
Enchondroma is a type of benign bone tumor belonging to the group of cartilage tumors. There may be no symptoms, or it may present typically in the short tubular bones of the hands with a swelling, pain or pathological fracture.
Diagnosis is ...
or
osteochondroma
Osteochondromas are the most common benign tumors of the bones.
The tumors take the form of cartilage-capped bony projections or outgrowth on the surface of bones exostoses. It is characterized as a type of overgrowth that can occur in any bone wh ...
. A small minority of secondary chondrosarcomas occur in people with
Maffucci syndrome
Maffucci syndrome is a very rare disorder in which multiple benign tumors of cartilage develop within the bones (such tumors are known as enchondromas). The tumors most commonly appear in the bones of the hands, feet, and limbs, causing bone defor ...
and
Ollier disease
Ollier disease is a rare sporadic nonhereditary skeletal disorder in which typically benign cartilaginous tumors ( enchondromas) develop near the growth plate cartilage. This is caused by cartilage rests that grow and reside within the metaphysis ...
.
It has been associated with faulty
isocitrate dehydrogenase
Isocitrate dehydrogenase (IDH) () and () is an enzyme that catalyzes the oxidative decarboxylation of isocitrate, producing alpha-ketoglutarate (α-ketoglutarate) and CO2. This is a two-step process, which involves oxidation of isocitrate (a ...
1 and 2 enzymes, which are also associated with
glioma
A glioma is a type of tumor that starts in the glial cells of the brain or the spine. Gliomas comprise about 30 percent of all brain tumors and central nervous system tumours, and 80 percent of all malignant brain tumours.
Signs and symptoms
...
s and
leukemia
Leukemia ( also spelled leukaemia and pronounced ) is a group of blood cancers that usually begin in the bone marrow and result in high numbers of abnormal blood cells. These blood cells are not fully developed and are called ''blasts'' or ...
s.
Diagnosis
Imaging studies – including
radiographs ("x-rays"),
computerized tomography (CT), and
magnetic resonance imaging
Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio wav ...
(MRI) – are often used to make a presumptive
diagnosis
Diagnosis is the identification of the nature and cause of a certain phenomenon. Diagnosis is used in many different disciplines, with variations in the use of logic, analytics, and experience, to determine "cause and effect". In systems enginee ...
of chondrosarcoma. However, a definitive diagnosis depends on the identification of malignant cancer cells producing cartilage in a
biopsy
A biopsy is a medical test commonly performed by a surgeon, interventional radiologist, or an interventional cardiologist. The process involves extraction of sample cells or tissues for examination to determine the presence or extent of a disea ...
specimen that has been examined by a
pathologist
Pathology is the study of the causes and effects of disease or injury. The word ''pathology'' also refers to the study of disease in general, incorporating a wide range of biology research fields and medical practices. However, when used in ...
. In a few cases, usually of highly anaplastic tumors,
immunohistochemistry
Immunohistochemistry (IHC) is the most common application of immunostaining. It involves the process of selectively identifying antigens (proteins) in cells of a tissue section by exploiting the principle of antibodies binding specifically to an ...
(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 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.
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, but in some cases amputation is unavoidable. Amputation of the
arm
In human anatomy, the arm refers to the upper limb in common usage, although academically the term specifically means the upper arm between the glenohumeral joint (shoulder joint) and the elbow joint. The distal part of the upper limb between ...
,
leg
A leg is a weight-bearing and locomotive anatomical structure, usually having a columnar shape. During locomotion, legs function as "extensible struts". The combination of movements at all joints can be modeled as a single, linear element c ...
,
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 serv ...
, or half of the
pelvis
The pelvis (plural pelves or pelvises) is the lower part of the trunk, between the abdomen and the thighs (sometimes also called pelvic region), together with its embedded skeleton (sometimes also called bony pelvis, or pelvic skeleton).
The ...
(called a
hemipelvectomy) 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 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
In medicine, proton therapy, or proton radiotherapy, is a type of particle therapy that uses a beam of protons to irradiate diseased tissue, most often to treat cancer. The chief advantage of proton therapy over other types of external bea ...
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
In medicine, proton therapy, or proton radiotherapy, is a type of particle therapy that uses a beam of protons to irradiate diseased tissue, most often to treat cancer. The chief advantage of proton therapy over other types of external bea ...
radiation can be useful in awkward locations to make surgery more effective.
Recent studies have shown that induction of
apoptosis
Apoptosis (from grc, ἀπόπτωσις, apóptōsis, 'falling off') is a form of programmed cell death that occurs in multicellular organisms. Biochemical events lead to characteristic cell changes ( morphology) and death. These changes inclu ...
in high-grade chondrosarcoma, both directly and by enhancement of response to
chemotherapy
Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs ( chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemother ...
and
radiation
In physics, radiation is the emission or transmission of energy in the form of waves or particles through space or through a material medium. This includes:
* ''electromagnetic radiation'', such as radio waves, microwaves, infrared, vi ...
, is a valid therapeutic strategy.
[Jamil N, Howie S, Salter DM. Therapeutic molecular targets in human chondrosarcoma .Int J Exp Pathol 2010; 91:387–93]
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, only 10% of patients will survive one year. 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.
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References
External links
*
{{Authority control
Osseous and chondromatous neoplasia
Rare cancers
Sarcoma