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'{{Use dmy dates|date=December 2017}} {{Use American English|date=December 2017}} {{for|the journal|Sarcoma (journal)}} {{Infobox medical condition (new) | name = Sarcoma | pronounce = | synonyms = Sarcomas, sarcomata | image = Nibib 030207 105309 sarcoma.jpg | caption = [[Optical coherence tomography|Optical Coherence Tomography]] (OCT) image of a sarcoma | field = [[Oncology]] | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }} A '''sarcoma''' is a [[cancer]] that arises from transformed [[Cell (biology)|cells]] of [[mesenchyme|mesenchymal]] origin. Thus, [[malignant tumor]]s made of [[cancellous bone]], [[cartilage]], [[fat]], [[muscle]], [[vascular]], or [[Haematopoiesis|hematopoietic]] tissues are, by definition, considered sarcomas. This is in contrast to a malignant tumor originating from [[epithelium|epithelial]] cells, which are termed [[carcinoma]]. Human sarcomas are quite rare. Common malignancies, such as [[Breast cancer|breast]], [[Colon cancer|colon]], and [[Lung cancer|lung]] cancer, are almost always carcinoma.<ref name="IARC">{{cite book |author=International Agency for Research on Cancer |title=Pathology and Genetics of Tumours of Soft Tissue and Bone |publisher=WHO Press |location=St. Louis |year=2002 |pages= |isbn=9789283224136 |oclc= |doi= |accessdate=}}</ref> The term is from the [[Ancient Greek|Greek]] [[wikt:σάρξ|σάρξ]] ''sarx'' meaning "flesh". == Types == ([[ICD-O]] codes are provided, where available, along with the relevant edition.) * [[Askin's tumor]] (8803/3) * [[Sarcoma botryoides]] * [[Chondrosarcoma]] (9220/3–9240/3) * [[Ewing's]] (9260/3)—[[Primitive neuroectodermal tumor|PNET]] (9473/3) * [[Malignant Hemangioendothelioma]] (9130/3) * [[Malignant Schwannoma]] (9560/3–9561/3) * [[Osteosarcoma]] (9180/3–9190/3) * [[Soft tissue sarcoma]]s, including: ** [[Alveolar soft part sarcoma]] (9581/3) ** [[Angiosarcoma]] (9120/3) ** [[Phyllodes tumor|Cystosarcoma Phyllodes]] ** [[Dermatofibrosarcoma]] protuberans (DFSP) (8832/3–8833/3) ** [[Desmoid Tumor]] (8821/1–8822/1) ** [[Desmoplastic small round cell tumor]] (8806/3) ** [[Epithelioid Sarcoma]] (8804/3) ** [[Extraskeletal chondrosarcoma]] (9220/3) ** [[Extraskeletal osteosarcoma]] (9180/3) ** [[Fibrosarcoma]] (8810/3) ** [[Gastrointestinal stromal tumor]] (GIST) ** [[Hemangiopericytoma]] (9150)(Also known as "solitary fibrous tumor". Only a subset of these tumors are classified as malignant.) ** [[Hemangiosarcoma]] (9120/3) (More commonly referred to as "angiosarcoma") ** [[Kaposi's sarcoma]] (9140/3) ** [[Leiomyosarcoma]] (8890/3–8896/3) ** [[Liposarcoma]] (8850/3–8858/3) ** [[Lymphangiosarcoma]] (9170–9175) ** [[Lymphoma|Lymphosarcoma]] (Not considered to be sarcomas) ** [[Malignant fibrous histiocytoma]] (8830/3)(This is an obsolete term that is no longer recognized by the World Health Organization. Many of these tumors would currently be classified as "undifferentiated pleomorphic sarcoma".) ** [[Malignant peripheral nerve sheath tumor]] (MPNST) ** [[Neurofibrosarcoma]] (9540/3) ** [[Plexiform Fibrohistiocytic Tumor]] (8835/3) ** [[Rhabdomyosarcoma]] (8900–8920) ** [[Synovial sarcoma]] (9040/3–9043/3) ** Undifferentiated pleomorphic sarcoma (previously referred to as [[Malignant fibrous histiocytoma]]) Surgery is important in the treatment of most sarcomas.<ref name=Morris05>{{cite journal |author=Morris, C |title= Malignant Fibrous Histiocytoma (MFH) |journal=ESUN |volume=2 |issue=2 |year=2005 |url=http://sarcomahelp.org/mfh.html| accessdate=2011-10-19}}</ref><ref name="van Praag et al 2017">{{cite journal |doi=10.1016/j.ejca.2017.06.032 |title=A prediction model for treatment decisions in high-grade extremity soft-tissue sarcomas: Personalised sarcoma care (PERSARC) |journal=European Journal of Cancer |volume=83 |pages=313–23 |year=2017 |last1=van Praag |first1=Veroniek M. |last2=Rueten-Budde |first2=Anja J. |last3=Jeys |first3=Lee M. |last4=Laitinen |first4=Minna |last5=Pollock |first5=Rob |last6=Aston |first6=Will |last7=Van De Hage |first7=Jos A. |last8=Dijkstra |first8=P.D. Sander |last9=Ferguson |first9=Peter C. |last10=Griffin |first10=Anthony M. |last11=Willeumier |first11=Julie J. |last12=Wunder |first12=Jay S. |last13=Van De Sande |first13=Michiel A.J. |last14=Fiocco |first14=Marta }}</ref> [[Limb-sparing techniques|Limb sparing surgery]], as opposed to amputation, can now be used to save the limbs of patients in at least 90% of extremity tumor cases.<ref name=Morris05/> Additional treatments, including chemotherapy and radiation therapy, may be administered before and/or after surgery.<ref name=Buecker05/><ref name="van Praag et al 2017"/> [[Chemotherapy]] significantly improves the prognosis for many sarcoma patients, especially those with bone sarcomas.<ref>{{cite journal |author=Baker, L |title= A Rose is a Rose or a Thorn is a Thorn |journal=ESUN |volume=3 |issue=5 |year=2006 |url=http://sarcomahelp.org/articles/chemotherapy-rose.html | accessdate=2011-10-19}}</ref> Treatment can be a long and arduous process, lasting about a year for many patients.<ref name=Buecker05/> *[[Liposarcoma]] treatment consists of surgical resection, with chemotherapy not being used outside of the investigative setting. [[Adjuvant]] radiotherapy may also be used after surgical excision for liposarcoma.<ref>{{EMedicine|article|1102007|Liposarcoma Treatment & Management|treatment}}</ref> *Rhabdomyosarcoma is treated with [[surgery]], [[radiotherapy]], and/or [[chemotherapy]].<ref>{{cite web |title=Rhabdomyosarcoma |publisher=Boston Children's Hospital |url=http://www.childrenshospital.org/az/Site1068/mainpageS1068P0.html}}</ref> The majority of rhabdomyosarcoma patients have a 50–85% survival rate.<ref>{{cite journal |author=Wexler, L |title= Rhabdomyosarcoma |journal=ESUN |volume=1 |issue=4 |year=2004 |url=http://sarcomahelp.org/rhabdomyosarcoma.html | accessdate=2011-10-19}}</ref> *Osteosarcoma is a tumor of the bone that is treated with surgical resection of as much of the cancer as possible, often along with [[neoadjuvant chemotherapy]].<ref>{{EMedicine|article|1256857|Osteosarcoma Treatment & Management|treatment}}</ref> Radiotherapy is a second alternative although not as successful. ==Diagnosis== ===Classification=== ====Tissue==== Sarcomas are given a number of different names based on the type of tissue that they most closely resemble. For example, [[osteosarcoma]] resembles [[bone]], [[chondrosarcoma]] resembles [[cartilage]], [[liposarcoma]] resembles [[fat]], and [[leiomyosarcoma]] resembles [[smooth muscle]]. ====Grade==== In addition to being named based on the tissue of origin, sarcomas are also assigned a grade (low, intermediate, or high) based on the presence and frequency of certain cellular and subcellular characteristics associated with malignant biological behavior. Low grade sarcomas are usually treated surgically, although sometimes [[radiation therapy]] or [[chemotherapy]] are used. Intermediate and high grade sarcomas are more frequently treated with a combination of [[surgery]], [[chemotherapy]] and/or [[radiation therapy]].<ref name=Buecker05>{{cite journal |author=Buecker, P |title=Sarcoma: A Diagnosis of Patience |journal=ESUN |volume=2 |issue=5 |year=2005 |url=http://sarcomahelp.org/articles/patience.html | accessdate=2009-04-15}}</ref> Since higher grade tumors are more likely to undergo [[metastasis]] (invasion and spread to locoregional and distant sites), they are treated more aggressively. The recognition that many sarcomas are sensitive to chemotherapy has dramatically improved the survival of patients. For example, in the era before chemotherapy, long-term survival for patients with localized osteosarcoma was only approximately 20%, but now has risen to 60–70%.<ref>{{cite journal |vauthors=Longhi A, Errani C, De Paolis M, Mercuri M, Bacci G |title=Primary bone osteosarcoma in the pediatric age: state of the art |journal=Cancer Treat Rev. |volume=32 |issue=6 |pages=423–36 |date=Oct 2006 |pmid=16860938 |doi=10.1016/j.ctrv.2006.05.005 |url=}}</ref> ==Management== {{Empty section|date=December 2017}} ==Epidemiology== Sarcomas are quite rare with only 15,000 new cases per year in the United States.<ref>{{cite journal |vauthors=Borden EC, Baker LH, Bell RS, etal |title=Soft tissue sarcomas of adults: state of the translational science |journal=Clin. Cancer Res. |volume=9 |issue=6 |pages=1941–56 |date=Jun 2003 |pmid=12796356 |url=http://clincancerres.aacrjournals.org/cgi/pmidlookup?view=long&pmid=12796356}}</ref> Sarcomas therefore represent about one percent of the 1.5 million new cancer diagnoses in that country each year.<ref>{{cite web |title=Cancer Facts & Figures 2009 |publisher=American Cancer Society |url=http://www.cancer.org/docroot/STT/content/STT_1x_Cancer_Facts__Figures_2009.asp?from=fast}}</ref> Sarcomas affect people of all ages. Approximately 50% of bone sarcomas and 20% of soft tissue sarcomas are diagnosed in people under the age of 35.<ref>{{cite journal |author=Darling, J |title=A Different View of Sarcoma Statistics |journal=ESUN |volume=4 |issue=6 |year=2007 |url=http://sarcomahelp.org/articles/sarcoma-statistics.html | accessdate=2012-10-06}}</ref> Some sarcomas, such as [[leiomyosarcoma]], [[chondrosarcoma]], and [[gastrointestinal stromal tumor]] (GIST), are more common in adults than in children. Most high-grade bone sarcomas, including [[Ewing's sarcoma]] and [[osteosarcoma]], are much more common in children and young adults. ==Awareness== In the US, July is widely recognized as Sarcoma Awareness Month.<ref>{{cite web |title=Cancer Awareness Dates |publisher=American Society of Clinical Oncology |url=http://www.cancer.net/research-and-advocacy/cancer-awareness-dates}}</ref> The UK has a Sarcoma Awareness Week July in led by [[Sarcoma UK]], the bone and soft tissue cancer charity.<ref>{{cite web |title=Sarcoma Awareness Week 2018 |publisher=Sarcoma UK |url=https://sarcoma.org.uk/get-involved/SAW|accessdate=13 April 2018}}</ref> ==References== {{Reflist}} == External links == {{Medical resources | DiseasesDB = | ICD10 = | ICD9 = | ICDO = {{ICDO|8800|3}} | OMIM = | MedlinePlus = | eMedicineSubj = | eMedicineTopic = | MeshID = D012509 }} * [http://www.cancer.gov/types/bone/hp National Cancer Institute] Bone sarcoma * {{DMOZ|Health/Conditions_and_Diseases/Cancer/Musculoskeletal/}} {{Soft tissue tumors and sarcomas |state=collapsed}} {{Osseous and chondromatous tumors |state=collapsed}} {{Vascular tumors |state=collapsed}} [[Category:Anatomical pathology]] [[Category:Soft tissue disorders]] [[Category:Sarcoma]]'