Chondrosarcoma

Chondrosarcoma
Chondrosarcoma
Classification and external resources

Histopathologic image of chondrosarcoma of the chest wall. Surgical resection of recurrent mass. H & E stain.
ICD-9 170.9
ICD-O: M9220/3-9240/3
OMIM 215300
DiseasesDB 2597
eMedicine article/388869
MeSH D002813

Chondrosarcoma is a cancer composed of cells derived from transformed cells that produce cartilage.[1] Chondrosarcoma is a member of a category of "soft tissue" malignancies known as sarcomas. About 30% of skeletal system cancers are chondrosarcomas.[2] 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.[3]

Contents

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 metastasis).

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.[4] 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:

Diagnosis

MRI of a Left Pelvis Chondrosarcoma in a 26yr male

Imaging studies - including radiographs ("x-rays"), computerized tomography (CT), and magnetic resonance imaging (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.

Causes

The cause is unknown. Patients may have a history of enchondroma or osteochondroma. A small minority of secondary chondrosarcomas occur in patients with Maffucci syndrome and Ollier disease.[5]

Treatment

Treatment depends on the location of the disease and the aggressiveness of the tumors.[4] 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, 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)

  • 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 is showing promise with local tumor control at over 80%.[6]

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.[7]

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.[3] 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.

Chondrosarcoma

  • Chondrosarcoma (2).jpg
  • Chondrosarcoma (3).jpg
  • Chondrosarcoma (4).jpg
  • Chondrosarcoma (5).jpg

References

  1. ^ Gelderblom H, Hogendoorn PC, Dijkstra SD, et al. (March 2008). "The clinical approach towards chondrosarcoma". Oncologist 13 (3): 320–9. doi:10.1634/theoncologist.2007-0237. PMID 18378543. http://theoncologist.alphamedpress.org/cgi/pmidlookup?view=long&pmid=18378543. [dead link]
  2. ^ http://www.aanos.org/epidemiologyboneca.html
  3. ^ a b "Chondrosarcoma: Overview - eMedicine Radiology". http://www.emedicine.com/radio/topic168.htm. Retrieved 2009-02-14. 
  4. ^ a b "Chondrosarcoma : Cancerbackup". http://www.cancerbacup.org.uk/Cancertype/Bone/Typesofbonecancer/Chondrosarcoma#3340. Retrieved 2009-02-14. 
  5. ^ Lin PP, Moussallem CD, Deavers MT. Secondary chondrosarcoma. J Am Acad Orthop Surg 2010;18):608-15.
  6. ^ "Proton Therapy at PSI (Protonentherapie)". http://p-therapie.web.psi.ch/e/klinikerfahrung.html. Retrieved 2009-02-14. 
  7. ^ Jamil N, Howie S, Salter DM. Therapeutic molecular targets in human chondrosarcoma .Int J Exp Pathol 2010; 91:387-93

External links

v · d · eConnective tissue neoplasm: Osseous and Chondromatous tumors (ICD-O 9180–9269) (C40–C41/D16, 170/213)
Diaphysis
Metaphysis
Epiphysis
Other/ungrouped

M: BON/CAR

anat(c/f/k/f, u, t/p, l)/phys/devp/cell

noco/cong/tumr, sysi/epon, injr

proc, drug(M5)

Wikimedia Foundation. 2010.

Игры ⚽ Поможем написать реферат

Look at other dictionaries:

  • chondrosarcoma — ▪ pathology       rare malignant tumour of bone formed from cartilage. Pain is the most common symptom. Primary chondrosarcomas arise from a small collection of cartilage cells; the secondary type develops slowly from a previously benign tumour… …   Universalium

  • chondrosarcoma — noun A type of bone cancer, a cartilage based tumour …   Wiktionary

  • chondrosarcoma — A malignant neoplasm derived from cartilage cells, occurring most frequently in pelvic bones or near the ends of long bones, in middle aged and older people; most chondrosarcomas arise de novo, but some may develop in a preexisting benign… …   Medical dictionary

  • chondrosarcoma — n. malignant cancerous growth in the cartilage …   English contemporary dictionary

  • chondrosarcoma — chon·dro·sarcoma …   English syllables

  • chondrosarcoma — n. an uncommon malignant tumour of cartilage cells occurring in a bone, most frequently in the femur, humerus, ribs, or pelvis, and most commonly affecting adults in their fifties and sixties. It has a typical ‘snowstorm’ appearance on X ray.… …   The new mediacal dictionary

  • chondrosarcoma — A type of cancer that forms in cartilage …   English dictionary of cancer terms

  • chondrosarcoma — noun a malignant neoplasm of cartilage cells • Hypernyms: ↑sarcoma * * * | ̷ ̷ ̷ ̷ at chondro + noun (plural chondrosarcomas also chondrosarcomata) Etymology …   Useful english dictionary

  • juxtacortical chondrosarcoma — chondrosarcoma arising in relation to the cortex of the periosteum, occurring on the surface of the bone, particularly the femur or humerus, usually in adults …   Medical dictionary

  • peripheral chondrosarcoma — chondrosarcoma arising on the surface of a bone; it may be either primary or secondary to underlying neoplasms, which are usually benign cartilage tumors, and generally presents as a large mass …   Medical dictionary

Share the article and excerpts

Direct link
… Do a right-click on the link above
and select “Copy Link”