Chondrosarcoma: Difference between revisions
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'''Chondrosarcoma''' is a [[bone sarcoma]], a primary [[cancer]] composed of cells derived from transformed cells that produce [[cartilage]].<ref name="pmid18378543">{{cite journal|vauthors=Gelderblom H, Hogendoorn PC, Dijkstra SD |title=The clinical approach towards chondrosarcoma |journal=Oncologist |volume=13 |issue=3 |pages=320–9 |date=March 2008 |pmid=18378543 |doi=10.1634/theoncologist.2007-0237 |s2cid=20484590 |display-authors=etal |doi-access=free }}</ref> A chondrosarcoma is a member of a category of tumors of bone and soft tissue known as [[sarcoma]]s. About 30% of bone sarcomas are chondrosarcomas.<ref>{{cite web |url=http://www.aanos.org/epidemiologyboneca.html |title=AANOS |access-date=2011-10-30 |url-status=dead |archive-url=https://web.archive.org/web/20120430183809/http://www.aanos.org/epidemiologyboneca.html |archive-date=2012-04-30 }}</ref> It is resistant to [[chemotherapy]] and [[radiotherapy]]. 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]] than the [[appendicular skeleton]].<ref name="urlChondrosarcoma: Overview - eMedicine Radiology">{{cite web |url=http://www.emedicine.com/radio/topic168.htm |title=Chondrosarcoma: Overview – eMedicine Radiology |access-date=2009-02-14}}</ref> | '''Chondrosarcoma''' is a [[bone sarcoma]], a primary [[cancer]] composed of cells derived from transformed cells that produce [[cartilage]].<ref name="pmid18378543">{{cite journal|vauthors=Gelderblom H, Hogendoorn PC, Dijkstra SD |title=The clinical approach towards chondrosarcoma |journal=Oncologist |volume=13 |issue=3 |pages=320–9 |date=March 2008 |pmid=18378543 |doi=10.1634/theoncologist.2007-0237 |s2cid=20484590 |display-authors=etal |doi-access=free }}</ref> A chondrosarcoma is a member of a category of tumors of bone and soft tissue known as [[sarcoma]]s. About 30% of bone sarcomas are chondrosarcomas.<ref>{{cite web |url=http://www.aanos.org/epidemiologyboneca.html |title=AANOS |access-date=2011-10-30 |url-status=dead |archive-url=https://web.archive.org/web/20120430183809/http://www.aanos.org/epidemiologyboneca.html |archive-date=2012-04-30 }}</ref> It is resistant to [[chemotherapy]] and [[radiotherapy]] with mesenchymal suptype being a notable exception.<ref>{{Cite journal |last1=Remiszewski |first1=Piotr |last2=Wąż |first2=Julia |last3=Falkowski |first3=Sławomir |last4=Rutkowski |first4=Piotr |last5=Czarnecka |first5=Anna M. |date=2025-11-03 |title=Chemotherapy Strategies and Their Efficacy for Mesenchymal Chondrosarcoma |journal=Current Oncology |language=en |volume=32 |issue=11 |pages=615 |doi=10.3390/curroncol32110615 |doi-access=free |issn=1718-7729}}</ref> 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]] than the [[appendicular skeleton]].<ref name="urlChondrosarcoma: Overview - eMedicine Radiology">{{cite web |url=http://www.emedicine.com/radio/topic168.htm |title=Chondrosarcoma: Overview – eMedicine Radiology |access-date=2009-02-14}}</ref> | ||
==Types== | ==Types== | ||
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| Periosteal (aka juxtacortical)<ref>{{Cite journal|last=Schajowicz|first=F.|date=November 1977|title=Juxtacortical chondrosarcoma|journal=The Journal of Bone and Joint Surgery. British Volume|volume=59-B|issue=4|pages=473–480|doi=10.1302/0301-620X.59B4.270475|issn=0301-620X|pmid=270475|doi-access=free}}</ref><ref name=":1">{{Cite book | | Periosteal (aka juxtacortical)<ref>{{Cite journal|last=Schajowicz|first=F.|date=November 1977|title=Juxtacortical chondrosarcoma|journal=The Journal of Bone and Joint Surgery. British Volume|volume=59-B|issue=4|pages=473–480|doi=10.1302/0301-620X.59B4.270475|issn=0301-620X|pmid=270475|doi-access=free}}</ref><ref name=":1">{{Cite book|title=Pathology and genetics of tumours of soft tissue and bone|date=2002|publisher=IARC Press|others=Fletcher, Christopher D. M., Unni, K. Krishnan, 1941–, Mertens, Fredrik., World Health Organization., International Agency for Research on Cancer.|isbn=978-92-832-2413-6|location=Lyon|oclc=51001831}}</ref> | ||
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[[File:Metastatic chondrosarcoma at the lower lip.jpg|thumb|Metastatic chondrosarcoma at the lower lip]] | [[File:Metastatic chondrosarcoma at the lower lip.jpg|thumb|Metastatic chondrosarcoma at the lower lip]] | ||
Imaging studies – including [[radiographs]] ("x-rays"), [[computerized tomography]] (CT), and [[magnetic resonance imaging]] (MRI) – are often used to make a presumptive [[diagnosis]] of chondrosarcoma.<ref>[http://www.claripacs.com/a.php?a=91 CT scan and gross pathology of a chondrosarcoma] 2012-12-18</ref> 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.{{ | Imaging studies – including [[radiographs]] ("x-rays"), [[computerized tomography]] (CT), and [[magnetic resonance imaging]] (MRI) – are often used to make a presumptive [[diagnosis]] of chondrosarcoma.<ref>[http://www.claripacs.com/a.php?a=91 CT scan and gross pathology of a chondrosarcoma] 2012-12-18</ref> 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.<ref>{{Cite journal |last1=Remiszewski |first1=Piotr |last2=Spałek |first2=Mateusz J. |last3=Koseła-Paterczyk |first3=Hanna |last4=Wągrodzki |first4=Michał |last5=Kalinowska |first5=Iwona |last6=Rutkowski |first6=Piotr |last7=Czarnecka |first7=Anna M. |date=2025-09-25 |title=Extraskeletal mesenchymal chondrosarcoma: oligometastatic disease and effect of multimodal treatment |url=https://journals.viamedica.pl/oncology_in_clinical_practice/article/view/108195 |journal=Oncology in Clinical Practice |doi=10.5603/ocp.108195 |issn=2450-6478|doi-access=free }}</ref> | ||
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.<ref>{{cite web |url=https://www.lecturio.com/concepts/chondrosarcoma/| title=Chondrosarcoma | website=The Lecturio Medical Concept Library |access-date= 8 July 2021}}</ref> | 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.<ref>{{cite web |url=https://www.lecturio.com/concepts/chondrosarcoma/| title=Chondrosarcoma | website=The Lecturio Medical Concept Library |access-date= 8 July 2021}}</ref> | ||
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.{{ | 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.<ref>{{Cite journal |last1=Albergo |first1=Jose I. |last2=Gaston |first2=Czar L. |last3=Jeys |first3=Lee M. |last4=Khajuria |first4=Arun |last5=Carter |first5=Simon R. |last6=Tillman |first6=Roger M. |last7=Abudu |first7=Adesegun T. |last8=Grimer |first8=Robert J. |date=May 2015 |title=Management and prognostic significance of pathological fractures through chondrosarcoma of the femur |url=http://link.springer.com/10.1007/s00264-015-2706-x |journal=International Orthopaedics |language=en |volume=39 |issue=5 |pages=943–946 |doi=10.1007/s00264-015-2706-x |pmid=25711397 |issn=0341-2695|url-access=subscription }}</ref> | ||
==Treatment== | ==Treatment== | ||
Treatment depends on the location of the disease and the aggressiveness of the tumors.<ref name="urlChondrosarcoma : Cancerbackup">{{cite web |url=http://www.cancerbacup.org.uk/Cancertype/Bone/Typesofbonecancer/Chondrosarcoma#3340 |title=Chondrosarcoma : Cancerbackup |access-date=2009-02-14 |archive-url=https://web.archive.org/web/20080905213942/http://www.cancerbacup.org.uk/Cancertype/Bone/Typesofbonecancer/Chondrosarcoma#3340 |archive-date=2008-09-05 |url-status=dead }}</ref> | Treatment depends on the location of the disease and the aggressiveness of the tumors.<ref name="urlChondrosarcoma : Cancerbackup">{{cite web |url=http://www.cancerbacup.org.uk/Cancertype/Bone/Typesofbonecancer/Chondrosarcoma#3340 |title=Chondrosarcoma : Cancerbackup |access-date=2009-02-14 |archive-url=https://web.archive.org/web/20080905213942/http://www.cancerbacup.org.uk/Cancertype/Bone/Typesofbonecancer/Chondrosarcoma#3340 |archive-date=2008-09-05 |url-status=dead }}</ref> | ||
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 techniques|limb-sparing operation]] can be performed, but 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.{{ | 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 techniques|limb-sparing operation]] can be performed, but 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.<ref>{{Cite journal |last1=Arnal-Burró |first1=J. |last2=Calvo-Haro |first2=J.A. |last3=Igualada-Blazquez |first3=C. |last4=Gil-Martínez |first4=P. |last5=Cuervo-Dehesa |first5=M. |last6=Vaquero-Martín |first6=J. |date=January 2016 |title=Hemipelvectomy for the treatment of high-grade sarcomas: Pronostic of chondrosarcomas compared to other histological types |url=https://linkinghub.elsevier.com/retrieve/pii/S1988885615000863 |journal=Revista Española de Cirugía Ortopédica y Traumatología (English Edition) |language=en |volume=60 |issue=1 |pages=67–74 |doi=10.1016/j.recote.2015.11.003|url-access=subscription }}</ref> | ||
There are two kinds of hemipelvectomy – internal and external. | There are two kinds of hemipelvectomy – internal and external. | ||
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Amputation at the hip is called hip disarticulation and amputees who have had this amputation are also called hip disartics. | 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%.<ref name="urlProton Therapy at PSI (Protonentherapie)">{{cite web |url=http://p-therapie.web.psi.ch/e/klinikerfahrung.html |title=Proton Therapy at PSI (Protonentherapie) |access-date=2009-02-14}}</ref> | Chemotherapy or traditional radiotherapy are not very effective for most chondrosarcomas, although [[proton therapy]] is showing promise with local tumor control at over 80%.<ref name="urlProton Therapy at PSI (Protonentherapie)">{{cite web |url=http://p-therapie.web.psi.ch/e/klinikerfahrung.html |title=Proton Therapy at PSI (Protonentherapie) |access-date=2009-02-14}}</ref> The only potentially chemosensitive chondrosarcoma subtypes are dedifferentiated and mesenchymal. <ref>{{Cite journal |last1=Remiszewski |first1=Piotr |last2=Wąż |first2=Julia |last3=Falkowski |first3=Sławomir |last4=Rutkowski |first4=Piotr |last5=Czarnecka |first5=Anna M. |date=2025-11-03 |title=Chemotherapy Strategies and Their Efficacy for Mesenchymal Chondrosarcoma |journal=Current Oncology |language=en |volume=32 |issue=11 |pages=615 |doi=10.3390/curroncol32110615 |doi-access=free |issn=1718-7729}}</ref><ref>{{Cite journal |last1=Biermann |first1=J. Sybil |last2=Chow |first2=Warren |last3=Reed |first3=Damon R. |last4=Lucas |first4=David |last5=Adkins |first5=Douglas R. |last6=Agulnik |first6=Mark |last7=Benjamin |first7=Robert S. |last8=Brigman |first8=Brian |last9=Budd |first9=G. Thomas |last10=Curry |first10=William T. |last11=Didwania |first11=Aarati |last12=Fabbri |first12=Nicola |last13=Hornicek |first13=Francis J. |last14=Kuechle |first14=Joseph B. |last15=Lindskog |first15=Dieter |date=February 2017 |title=NCCN Guidelines Insights: Bone Cancer, Version 2.2017 |url=https://jnccn.org/doi/10.6004/jnccn.2017.0017 |journal=Journal of the National Comprehensive Cancer Network |language=en |volume=15 |issue=2 |pages=155–167 |doi=10.6004/jnccn.2017.0017 |pmid=28188186 |issn=1540-1405}}</ref> | ||
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. | 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. | ||
Latest revision as of 03:02, 8 December 2025
Template:Short description Template:Infobox medical condition (new) Chondrosarcoma is a bone sarcoma, a primary cancer composed of cells derived from transformed cells that produce cartilage.[1] A chondrosarcoma is a member of a category of tumors of bone and soft tissue known as sarcomas. About 30% of bone sarcomas are chondrosarcomas.[2] It is resistant to chemotherapy and radiotherapy with mesenchymal suptype being a notable exception.[3] 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 than the appendicular skeleton.[4]
Types
| Subtype | Percentage of cases % | |
|---|---|---|
| Conventional | Primary central | ~75%[5] |
| Secondary peripheral | ~10%[5] | |
| Periosteal (aka juxtacortical)[6][7] | <1%[5] | |
| Rare | Dedifferentiated | ~10%[5] |
| Mesenchymal | ~2%[5] | |
| Clear-cell | ~2%[7] |
Symptoms and signs
Causes
The cause is unknown. There may be a history of enchondroma or osteochondroma. A small minority of secondary chondrosarcomas occur in people with Maffucci syndrome and Ollier disease.[8]
It has been associated with faulty isocitrate dehydrogenase 1 and 2 enzymes, which are also associated with gliomas and leukemias.[9]
Diagnosis
Imaging studies – including radiographs ("x-rays"), computerized tomography (CT), and magnetic resonance imaging (MRI) – are often used to make a presumptive diagnosis of chondrosarcoma.[10] 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.[11]
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.[12]
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.[13]
Treatment
Treatment depends on the location of the disease and the aggressiveness of the tumors.[14]
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, leg, jaw, or half of the pelvis (called a hemipelvectomy) may be necessary in some cases.[15]
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 is showing promise with local tumor control at over 80%.[16] The only potentially chemosensitive chondrosarcoma subtypes are dedifferentiated and mesenchymal. [17][18]
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.[19]
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.[4] 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.[20]
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References
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- ↑ CT scan and gross pathology of a chondrosarcoma 2012-12-18
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- ↑ Jamil N, Howie S, Salter DM. Therapeutic molecular targets in human chondrosarcoma .Int J Exp Pathol 2010; 91:387–93
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External links
Template:Medical resources Script error: No such module "Navbox". Template:Osseous and chondromatous tumors Template:Authority control