Editing Chondrosarcoma

{{Short description|Malignant tumor originating in cartilage}}
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| name            = Chondrosarcoma
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| image           = Chondrosarcoma (1).jpg
| caption         = Histopathologic image of chondrosarcoma of the chest wall. Surgical resection of recurrent mass. H & E stain.
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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>

==Types==
{| class="wikitable"
|+Table 1:  Types of chondrosarcoma
|- valign=top
!
! <u>Subtype</u>
! Percentage of cases %
|- valign=top
| rowspan="3" | '''Conventional'''
| Primary central
| ~75%<ref name=":0">{{Cite journal|last1=Gelderblom|first1=Hans|last2=Hogendoorn|first2=Pancras C. W.|last3=Dijkstra|first3=Sander D.|last4=van Rijswijk|first4=Carla S.|last5=Krol|first5=Augustinus D.|last6=Taminiau|first6=Antonie H. M.|last7=Bovée|first7=Judith V. M. G.|date=March 2008|title=The clinical approach towards chondrosarcoma|journal=The Oncologist|volume=13|issue=3|pages=320–329|doi=10.1634/theoncologist.2007-0237|issn=1083-7159|pmid=18378543|s2cid=20484590 |doi-access=free}}</ref>
|-
|Secondary peripheral
|~10%<ref name=":0" />
|- valign="TOP"
| 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|url=https://www.worldcat.org/oclc/51001831|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>
| <1%<ref name=":0" />
|- valign="top"
| rowspan="3" |'''Rare'''
| Dedifferentiated
| valign="bottom" | ~10%<ref name=":0" />
|- valign="top"
| Mesenchymal
| ~2%<ref name=":0" />
|- valign="TOP"
| Clear-cell
| ~2%<ref name=":1" />
|}

==Symptoms and signs==
* Back or thigh pain
* [[Sciatica]]
* Bladder symptoms
* Unilateral [[edema]]

==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]].<ref>{{Cite journal|title = Secondary chondrosarcoma|journal = The Journal of the American Academy of Orthopaedic Surgeons|date = Oct 2010|issn = 1067-151X|pmid = 20889950|pages = 608–615|volume = 18|issue = 10|first1 = Patrick P.|last1 = Lin|first2 = Charbel D.|last2 = Moussallem|first3 = Michael T.|last3 = Deavers|doi = 10.5435/00124635-201010000-00004|s2cid = 25873835}}</ref>

It has been associated with faulty [[isocitrate dehydrogenase]] 1 and 2 enzymes, which are also associated with [[glioma]]s and [[leukemia]]s.<ref>{{Cite journal|title = IDH1 and IDH2 mutations are frequent events in central chondrosarcoma and central and periosteal chondromas but not in other mesenchymal tumours|journal = The Journal of Pathology|date = Jul 2011|issn = 1096-9896|pmid = 21598255|pages = 334–343|volume = 224|issue = 3|doi = 10.1002/path.2913|first1 = M. Fernanda|last1 = Amary|first2 = Krisztian|last2 = Bacsi|first3 = Francesca|last3 = Maggiani|first4 = Stephen|last4 = Damato|first5 = Dina|last5 = Halai|first6 = Fitim|last6 = Berisha|first7 = Robin|last7 = Pollock|first8 = Paul|last8 = O'Donnell|first9 = Anita|last9 = Grigoriadis|s2cid = 24201208|hdl = 1887/108488|hdl-access = free}}</ref>

==Diagnosis==
[[File:mrichondrosarcoma.jpg|right|thumb|MRI of a left-pelvis chondrosarcoma in a 26-year-old male]]

[[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.{{cn|date=October 2020}}

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.{{cn|date=October 2020}}

==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>  

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.{{cn|date=October 2020}}

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%.<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>

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.<ref name="JamilHowie">Jamil N, Howie S, Salter DM. Therapeutic molecular targets in human chondrosarcoma .Int J Exp Pathol 2010; 91:387–93</ref>

==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.<ref name="urlChondrosarcoma: Overview - eMedicine Radiology"/>  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.<ref>{{cite journal |vauthors= Limaiem F, Davis D, Sticco K |url=https://www.ncbi.nlm.nih.gov/books/NBK538132/| title=Chondrosarcoma
|website=National Center for Biotechnology Information, U.S. National Library of Medicine |year=2021|pmid=30844159|access-date= 8 July 2021}}</ref>

<gallery>
Image:Chondrosarcoma (2).jpg
Image:Chondrosarcoma (3).jpg
Image:Chondrosarcoma (4).jpg
Image:Chondrosarcoma (5).jpg
</gallery>

==References==
{{Reflist}}

==External links==
* {{Commons category-inline}}
{{Medical resources
|  DiseasesDB      = 2597
|  ICD10           =
|  ICD9            = {{ICD9|170.9}}
|  ICDO            = {{ICDO|9220|3}}-9240/3
|  OMIM            = 215300
|  MedlinePlus     =
|  eMedicineSubj   = article
|  eMedicineTopic  = 388869
|  MeshID          = D002813
}}
{{Medicine|state=collapsed}}
{{Osseous and chondromatous tumors}}
{{Authority control}}

[[Category:Osseous and chondromatous neoplasia]]
[[Category:Rare cancers]]
[[Category:Sarcoma]]