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'{{Short description|Type of malignant tumor (cancer)}} {{cs1 config|name-list-style=vanc|display-authors=6}} {{for|the journal|Sarcoma (journal)}} {{Use dmy dates|date=December 2017}} {{Use American English|date=December 2017}} {{Infobox medical condition (new) | name = Sarcoma | pronounce = | synonyms = Sarcomas, sarcomata | image = Nibib 030207 105309 sarcoma.jpg | caption = [[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 [[malignant tumor]], a type of [[cancer]] that arises from [[Cell (biology)|cells]] of [[mesenchyme|mesenchymal]] ([[connective tissue]]) origin.<ref>{{cite journal | vauthors = Yang J, Ren Z, Du X, Hao M, Zhou W | title = The role of mesenchymal stem/progenitor cells in sarcoma: update and dispute | journal = Stem Cell Investigation | volume = 1 | pages = 18 | date = 2014-10-27 | pmid = 27358864 | pmc = 4923508 | doi = 10.3978/j.issn.2306-9759.2014.10.01 }}</ref><ref name=":1" /> Connective tissue is a broad term that includes [[cancellous bone|bone]], [[cartilage]], [[Body fat|fat]], [[vascular]], or [[Haematopoiesis|other structural]] tissues, and sarcomas can arise in any of these types of tissues.<ref name=":1" /><ref name=":2" /> As a result, there are many subtypes of sarcoma, which are classified based on the specific tissue and type of cell from which the tumor originates.<ref name=":0" /> Sarcomas are ''primary'' connective tissue tumors, meaning that they arise in connective tissues.<ref name=":1" /> This is in contrast to ''secondary'' (or "metastatic") connective tissue tumors, which occur when a cancer from elsewhere in the body (such as the lungs, breast tissue or prostate) spreads to the connective tissue.<ref>{{Cite web|url=https://www.cancer.gov/types/metastatic-cancer|title=Metastatic Cancer|date=2015-05-12|website=National Cancer Institute|language=en|access-date=2019-03-22}}</ref> Sarcomas are one of five different [[List of cancer types|types of cancer]], classified by the [[list of distinct cell types in the adult human body|cell type]] from which they originate.<ref name=def>{{cite web|title=Defining Cancer| url=http://www.cancer.gov/cancertopics/cancerlibrary/what-is-cancer|website=National Cancer Institute|date=17 September 2007|access-date=10 June 2014}}</ref> The word ''sarcoma'' is derived from the [[Ancient Greek|Greek]] {{Lang|grc|σάρκωμα}} {{Lang|grc-latn|sarkōma}} 'fleshy excrescence or substance', itself from [[wikt:σάρξ|σάρξ]] {{Lang|grc-latn|sarx}} meaning 'flesh'.<ref>{{LSJ|sa/rkwma|σάρκωμα}}, {{LSJ|sa/rc|σάρξ|ref}}.</ref><ref>{{Cite web|url=https://www.merriam-webster.com/dictionary/sarcoma|title=Definition of SARCOMA|website=www.merriam-webster.com|language=en|access-date=2019-03-22}}</ref><ref>{{OEtymD|sarcoma}}</ref> == Classification == Sarcomas are typically divided into two major groups: [[bone sarcoma]]s and [[soft-tissue sarcoma]]s,<ref name=":1">{{Cite book|title=Cancer and its Management | edition = Seventh | vauthors = Tobias J |publisher=John Wiley & Sons, Ltd.|year=2015|isbn=978-1-118-46875-3|location=Chichester, West Sussex, PO198SQ, UK|pages=446}}</ref> each of which has multiple subtypes. In the United States, the American Joint Committee on Cancer (AJCC) publishes guidelines that classify the subtypes of sarcoma.<ref name=":0">{{Cite book|title=AJCC Cancer Staging Manual, Eight Edition| vauthors = Amin MB |publisher=Springer International Publishing AG Switzerland|year=2017|isbn=978-3-319-40617-6|location=Chicago, IL|pages=471–548}}</ref> These subtypes are as follows: ===Subtypes of bone sarcoma=== * [[Osteosarcoma]] * [[Chondrosarcoma]] * Poorly differentiated round/spindle cell tumors (includes [[Ewing sarcoma]]) * [[Hemangioendothelioma]] * [[Angiosarcoma]] * [[Fibrosarcoma]]/myofibrosarcoma * [[Chordoma]] * [[Adamantinoma]] * Other: ** [[Liposarcoma]] ** [[Leiomyosarcoma]] ** [[Malignant peripheral nerve sheath tumor]] ** [[Rhabdomyosarcoma]] ** [[Synovial sarcoma]] ** Malignant solitary fibrous tumor.<ref name=":0" /> ===Subtypes of soft-tissue sarcoma=== * [[Liposarcoma]] (includes the following varieties: [[Liposarcoma#Atypical lipomatous tumor/well-differentiated liposarcoma|atypical lipomatous tumor/well-differentiated liposarcoma]], [[Liposarcoma#Dedifferentiated liposarcoma|dedifferentiated liposarcoma]], [[Liposarcoma#Myxoid liposarcoma|myxoid sarcoma]], [[Liposarcoma#Pleomorphic liposarcoma|pleomorphic liposarcoma]], and [[Liposarcoma#Myxoid pleomorphic liposarcoma|myxoid pleomorphic liposarcoma]] * Atypical lipomatous tumor * [[Dermatofibrosarcoma protuberans]] (includes pigmented varieties) * [[Dermatofibrosarcoma protuberans, fibrosarcomatous]] * [[Giant cell fibroblastoma]] * Malignant [[solitary fibrous tumor]] * [[Inflammatory myofibroblastic tumour|Inflammatory myofibroblastic tumor]] * [[Low-grade myofibroblastic sarcoma]] * [[Fibrosarcoma]] (includes adult and sclerosing epithelioid varieties) * Myxofibrosarcoma (formerly myxoid malignant fibrous histiocytoma) * [[Low-grade fibromyxoid sarcoma]] * Giant cell tumor of soft tissues * [[Leiomyosarcoma]] * Malignant [[glomus tumor]] * [[Rhabdomyosarcoma]] (includes the following varieties: embryonal, alveolar, pleomorphic, and spindle cell/sclerosing) * [[Hemangioendothelioma]] (includes the following varieties: retiform, pseudomyogenic, and epithelioid) * [[Angiosarcoma]] of soft tissue * Extraskeletal osteosarcoma * [[Gastrointestinal stromal tumor]], malignant (GIST) * [[Malignant peripheral nerve sheath tumor]] (includes epithelioid variety) * Malignant Triton tumor * Malignant [[granular cell tumor]] * Malignant ossifying fibromyxoid tumor * Stromal sarcoma not otherwise specified * Myoepithelial carcinoma * Malignant phosphaturic mesenchymal tumor * [[Synovial sarcoma]] (includes the following varieties: spindle cell, biphasic, and not otherwise specified) * [[Epithelioid sarcoma]] * [[Alveolar soft part sarcoma]] * Clear cell sarcoma of soft tissue * Extraskeletal myxoid chondrosarcoma * Extraskeletal Ewing sarcoma * [[Interdigitating dendritic cell sarcoma]] * [[Desmoplastic small-round-cell tumor|Desmoplastic small round cell tumor]] * Extrarenal rhabdoid tumor * [[Perivascular epithelioid cell tumour|Perivascular epithelioid cell tumor]], not otherwise specified * Intimal sarcoma * Undifferentiated [[spindle cell sarcoma]] * Undifferentiated pleomorphic sarcoma * Undifferentiated round cell sarcoma * Undifferentiated epithelioid sarcoma * Undifferentiated sarcoma, not otherwise specified.<ref name=":0" /> == Signs and symptoms == Symptoms of bone sarcomas typically include bone pain, especially at night, and swelling around the site of the tumor.<ref name=":1" /> Symptoms of [[soft-tissue sarcoma]]s vary, but they often present as firm, often times painless lumps or nodules.<ref name=":1" /> Gastrointestinal stromal tumors (GIST, a subtype of soft-tissue sarcoma) often are asymptomatic, but can be associated with vague complaints of abdominal pain, bleeding into the intestines, a feeling of fullness, or other signs of intestinal obstruction.<ref name=":1" /> ==Cause== === Causes and risk factors === The cause of most '''bone sarcomas''' is not known,<ref name=":2">{{Cite book|title=DeVita, Hellman and Rosenberg's Cancer: Principles & Practice of Oncology | edition = 10th| vauthors = DeVita Jr V |publisher=Wolters Kluwer Health|year=2015|isbn=978-1-4511-9294-0|location=Philadelphia, PA|pages=1241–1313}}</ref> but several factors are associated with an increased risk of developing bone sarcoma. Previous exposure to ionizing radiation (such as prior radiation therapy) is one such risk factor.<ref name=":1" /> Therapeutic radiation is associated with sarcoma after 10 to 20 years.<ref>{{Cite web |title=UpToDate |url=https://www.uptodate.com/contents/radiation-associated-sarcomas#! |access-date=2023-03-19 |website=www.uptodate.com}}</ref> Exposure to alkylating agents, such as those found in [[Alkylating antineoplastic agent|certain cancer chemotherapeutic medicines]], also increases the risk of bone sarcoma.<ref name=":2" /> Certain inherited genetic syndromes, including [[Li–Fraumeni syndrome|Li-Fraumeni syndrome]], inherited ''RB1'' gene mutations, and [[Paget's disease of bone]] are associated with an increased risk of developing bone sarcomas.<ref name=":1" /> Most '''soft-tissue sarcomas''' arise from what doctors call "sporadic" (or random) genetic mutations within an affected person's cells.<ref name=":2" /> Nevertheless, there are certain risk factors associated with an increased risk of developing soft-tissue sarcoma. Previous exposure to [[ionizing radiation]] is one such risk factor.<ref name=":1" /> Exposure to vinyl chloride (e.g., such as the fumes encountered in the production of [[polyvinyl chloride]] (PVC)), [[arsenic]] and [[Thorotrast]] all are associated with an increased risk of angiosarcoma.<ref name=":1" /><ref name=":2" /> Lymphedema, such as that resulting from certain types of breast cancer treatment, also is a risk factor for development of angiosarcoma.<ref name=":2" /> As with bone sarcomas, certain inherited genetic syndromes also are associated with an increased risk of developing soft-tissue sarcoma, including [[Li–Fraumeni syndrome|Li-Fraumeni syndrome]], [[familial adenomatous polyposis]], [[Neurofibromatosis type I|neurofibromatosis type 1]], and heritable ''RB1'' gene mutations.<ref name=":2" /> [[Kaposi's sarcoma|Kaposi sarcoma]] is caused by [[Kaposi's sarcoma-associated herpesvirus|Kaposi sarcoma-associated herpesvirus]] (HHV-8). == Mechanisms == The mechanisms by which healthy cells transform into cancer cells are described in detail elsewhere (see [[Cancer]] main page; [[Carcinogenesis]] main page). The precise molecular changes that result in sarcoma are not always known, but certain types of sarcomas are associated with particular genetic mutations.<ref name=":1" /><ref name=":2" /> Examples include: * Most cases of Ewing sarcoma are associated with a [[chromosomal translocation]] in which part of [[chromosome 11]] fuses with part of [[chromosome 22]].<ref name=":1" /> This results in the [[Ewing sarcoma breakpoint region 1|''EWSR1'' gene]] becoming fused to other genes, including the [[FLI1|''FLI1'' gene]] in 90% of Ewing cases and [[ERG (gene)|''ERG'' gene]] in 5-10% of cases.<ref name=":1" /> These fusions result in the production of abnormal proteins, although how these abnormal proteins result in cancer is not fully known.<ref name=":1" /> * Dermatofibrosarcoma protuberans often is associated with a chromosomal translocation in which the [[Collagen, type I, alpha 1|''COL1A1'' gene]] becomes fused to the [[PDGFRB|''PDGFRB'' gene]].<ref name=":2" /> This results in over-active [[Platelet-derived growth factor|PDGF]] signaling, which is thought to promote cell division and ultimately lead to tumor development.<ref name=":2" /> * Inflammatory myofibroblastic tumor often is associated with rearrangements of the [[Anaplastic lymphoma kinase|''ALK'' gene]], and occasionally with rearrangements of the [[HMGA2|''HMGA2'' gene]].<ref name=":2" /> * Tenosynovial giant cell tumor (not a sarcoma, but a non-metastasizing and locally aggressive soft tissue tumor) frequently is associated with a chromosomal translocation between [[chromosome 1]] and [[chromosome 2]], in which the [[Macrophage colony-stimulating factor|''CSF1'' gene]] becomes fused with the [[Collagen, type VI, alpha 3|''COL6A3'' gene]].<ref name=":2" /> This results in increased CSF1 protein production, which is thought to play a role in cancer development.<ref name=":2" /> * Many liposarcomas are associated with amplification of part of chromosome 12, which results in extra copies of known cancer-promoting genes ("[[oncogene]]s") such as the [[Cyclin-dependent kinase 4|''CDK4'' gene]], the [[Mdm2|''MDM2'' gene]] and the [[HMGA2|''HMGA2'' gene]].<ref name=":2" /> ==Diagnosis== ===Bone sarcomas=== {{Main|Bone sarcoma}} Diagnosis of [[bone sarcoma]]s begins with a thorough history and physical examination which may reveal characteristic signs and symptoms (see Signs and Symptoms above).<ref name=":2" /> Laboratory studies are not particularly useful in diagnosis, although some bone sarcomas (such as osteosarcoma) may be associated with elevated [[alkaline phosphatase]] levels, while others (such as Ewing sarcoma) can be associated with elevated [[erythrocyte sedimentation rate]].<ref name=":3">{{Cite book|title=Dahlin's Bone Tumors| vauthors = Unni K |publisher=Lippincott Williams & Wilkins|year=2010|isbn=978-0-7817-6242-7|location=Philadelphia, PA|pages=1–8}}</ref> Importantly, however, none of these laboratory findings are specific to bone sarcomas, meaning that elevations in these lab values are associated with many other conditions as well as sarcoma, and thus cannot be relied upon to conclusively diagnose sarcoma.<ref name=":2" /> Imaging studies are critically important in diagnosis, and most clinicians will order a plain [[Radiography|radiograph]] (X-ray) initially.<ref name=":2" /> Other imaging studies commonly used in diagnosis include [[magnetic resonance imaging]] (MRI) studies and [[Bone scintigraphy|radioisotope bone scans]].<ref name=":3" /><ref name=":2" /> A [[CT scan]] is typically not used in diagnosis of most types of bone sarcoma, although it is an important tool for staging (see below).<ref name=":2" /> Definitive diagnosis requires biopsy of the tumor and careful review of the biopsy specimen by an experienced pathologist.<ref name=":2" /> ===Soft-tissue sarcomas=== {{Main|Soft-tissue sarcoma}} Diagnosis of [[soft-tissue sarcoma]]s also begins with a thorough history and physical examination.<ref name=":2" /> Imaging studies can include either CT or MRI, although CT tends to be preferred for soft-tissue sarcomas located in the [[thorax]], [[abdomen]], or [[Retroperitoneal space|retroperitoneum]].<ref name=":2" /> [[Positron emission tomography]] (PET) also may be useful in diagnosis, although its most common use is for staging (see below).<ref name=":2" /> As with bone sarcomas, definitive diagnosis requires biopsy of the tumor with evaluation of histology by a trained pathologist.<ref name=":2" /><ref>{{Cite journal| vauthors = Rastogi S, Aggarwal A, Shishak S, Barwad A, Dhamija E, Pandey R, Mridha AR, Khan SA, Deo SS, Sharma MC |date=2019-08-09|title=Discordance of Histo-pathological Diagnosis of Patients with Soft Tissue Sarcoma Referred to Tertiary Care Center |url= http://waocp.com/journal/index.php/apjcc/article/view/274 |journal= Asian Pacific Journal of Cancer Care|volume=4 |issue=4 |pages=119–123 |doi=10.31557/apjcc.2019.4.4.119-123 |doi-access=free }}</ref> === Staging === In general, [[cancer staging]] refers to how advanced a cancer is, and usually it is based upon factors such as tumor size and whether it has spread to other parts of the body.<ref name=":2" /><ref>{{Cite web|url=https://www.cancer.gov/about-cancer/diagnosis-staging/staging|title=Staging|date=2015-03-09|website=National Cancer Institute|language=en|access-date=2019-03-21}}</ref> Staging is important because the stage affects the [[prognosis]] (likely outcome), as well as the types of treatments that are likely to be effective against the cancer.<ref name=":1" /><ref name=":0" /> With sarcomas, staging requires a determination of whether the tumor has grown into surrounding tissues ("local invasion"), as well as imaging to determine whether it has spread (a process known as "[[metastasis]]") to lymph nodes (forming "nodal metastases") or to other tissues or organs in the body (forming "distant metastases").<ref name=":0" /> The most common imaging tools used for staging '''bone sarcomas''' are MRI or CT to evaluate the primary tumor, contrast-enhanced CT of the chest to evaluate whether the cancer has spread (i.e., metastasized) to the lungs, and radioisotope bone scan to evaluate whether the cancer has spread to other bones.<ref name=":0" /> Staging for '''soft-tissue sarcomas''' typically includes imaging of the primary tumor by MRI or CT to determine tumor size, as well as contrast-enhanced CT of the chest to evaluate for metastatic tumors in the lungs.<ref name=":0" /> ===Grade=== Like some other cancers, sarcomas are assigned a [[Grading (tumors)|grade]] (low, intermediate, or high) based on the appearance of the tumor cells under a microscope.<ref name=":6">{{Cite web|url=https://www.cancer.gov/about-cancer/diagnosis-staging/prognosis/tumor-grade-fact-sheet|title=Tumor Grade|date=2013-05-09|website=National Cancer Institute|language=en|access-date=2019-03-21}}</ref> In general, grade refers to how aggressive the cancer is and how likely it is to spread to other parts of the body ("metastasize").<ref name=":6" /> Low-grade sarcomas have a better prognosis than higher-grade sarcomas, and are usually treated surgically, although sometimes radiation therapy or chemotherapy are used.<ref name=":2" /><ref name=":0" /> Intermediate- and high-grade sarcomas are more frequently treated with a combination of surgery, chemotherapy, or radiation therapy.<ref name=Buecker05>{{cite journal |vauthors=Buecker P |title=Sarcoma: A Diagnosis of Patience |journal=ESUN |volume=2 |issue=5 |year=2005 |url=http://sarcomahelp.org/articles/patience.html |access-date=2009-04-15 |archive-date=4 December 2020 |archive-url=https://web.archive.org/web/20201204224156/http://sarcomahelp.org/articles/patience.html |url-status=dead }}</ref> Since high-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 pediatric patients with localized osteosarcoma was only about 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 Treatment Reviews | volume = 32 | issue = 6 | pages = 423–436 | date = October 2006 | pmid = 16860938 | doi = 10.1016/j.ctrv.2006.05.005 }}</ref> == Screening == In the US, the US Preventive Services Task Force (USPSTF) publishes guidelines recommending [[Cancer screening|preventive screening]] for certain types of common cancers and other diseases.<ref name=":4">{{Cite web|url=https://www.uspreventiveservicestaskforce.org/BrowseRec/Index|title=Published Recommendations - US Preventive Services Task Force|website=www.uspreventiveservicestaskforce.org|access-date=2019-03-20}}</ref> {{As of|2019|March}}, the USPSTF does not recommend screening for sarcoma,<ref name=":4" /> possibly because it is a very rare type of cancer (see Epidemiology below). The American Cancer Society (ACS) also publishes guidelines recommending preventive screening for certain types of common cancers.<ref name=":5">{{Cite web|url=https://www.cancer.org/healthy/find-cancer-early/cancer-screening-guidelines/american-cancer-society-guidelines-for-the-early-detection-of-cancer.html|title=Cancer Screening Guidelines {{!}} Detecting Cancer Early|website=www.cancer.org|language=en|access-date=2019-03-20}}</ref> Like the USPSTF, {{As of|2019|March|lc=y}} ACS does not recommend preventive screening for sarcoma.<ref name=":5" /> However, patients with some inherited conditions, such as [[Neurofibromatosis type I|neurofibromatosis]], may benefit from screening for development of cancers from pre-existing benign tumors called neurofibromas. == Treatment == [[Surgery]] is the most common form of the treatment for most sarcomas that have not spread to other parts of the body, and for most sarcomas, surgery is the only curative treatment.<ref name=":2" /><ref name="Morris05">{{cite journal|vauthors=Morris C|year=2005|title=Malignant Fibrous Histiocytoma (MFH)|url=http://sarcomahelp.org/mfh.html|journal=ESUN|volume=2|issue=2|access-date=2011-10-19|archive-date=11 November 2012|archive-url=https://web.archive.org/web/20121111000838/http://sarcomahelp.org/mfh.html|url-status=dead}}</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 (arm or leg) sarcoma cases.<ref name="Morris05" /> Additional treatments, including [[chemotherapy]], [[radiation therapy]] (also called "radiotherapy"), which includes [[proton therapy]], may be administered ''before surgery'' (called "[[Neoadjuvant therapy|neoadjuvant]]" chemotherapy or radiotherapy) or ''after surgery'' (called "[[Adjuvant therapy|adjuvant]]" chemotherapy or radiotherapy).<ref name=":2" /><ref name="Buecker05" /> The use of neoadjuvant or adjuvant chemotherapy and radiotherapy significantly improves the prognosis for many sarcoma patients.<ref name=":2" /><ref>{{cite journal|vauthors=Baker L|year=2006|title=A Rose is a Rose or a Thorn is a Thorn|url=http://sarcomahelp.org/articles/chemotherapy-rose.html|journal=ESUN|volume=3|issue=5|access-date=2011-10-19|archive-date=19 January 2013|archive-url=https://web.archive.org/web/20130119081916/http://sarcomahelp.org/articles/chemotherapy-rose.html|url-status=dead}}</ref> Treatment can be a long and arduous process, lasting about a year for many patients.<ref name="Buecker05" /> * [[Liposarcoma]] treatment usually consists of surgical resection, with chemotherapy considered depending on the aggressiveness of the sarcoma. Radiotherapy may also be used before or after surgical excision for liposarcoma.<ref>{{EMedicine|article|1102007|Liposarcoma Treatment & Management|treatment}}</ref> * Pediatric rhabdomyosarcoma is usually treated with chemotherapy, surgery, and sometimes radiotherapy.<ref>{{cite web|url=http://www.childrenshospital.org/az/Site1068/mainpageS1068P0.html|title=Rhabdomyosarcoma|publisher=Boston Children's Hospital|access-date=2 March 2010|archive-date=23 May 2011|archive-url=https://web.archive.org/web/20110523150444/http://www.childrenshospital.org/az/Site1068/mainpageS1068P0.html|url-status=dead}}</ref> Pediatric rhabdomyosarcoma patients have a 50–85% long term survival rate.<ref>{{cite journal|vauthors=Wexler L|year=2004|title=Rhabdomyosarcoma|url=http://sarcomahelp.org/rhabdomyosarcoma.html|journal=ESUN|volume=1|issue=4|access-date=2011-10-19|archive-date=27 October 2012|archive-url=https://web.archive.org/web/20121027025134/http://sarcomahelp.org/rhabdomyosarcoma.html|url-status=dead}}</ref> * Osteosarcoma is a cancer of the bone that is treated with surgical resection of as much of the cancer as possible, often along with [[neoadjuvant chemotherapy|chemotherapy]].<ref>{{EMedicine|article|1256857|Osteosarcoma Treatment & Management|treatment}}</ref> Radiotherapy is a second alternative to surgery, although not as successful. It was believed that higher doses of chemotherapy might improve survival. However, high doses of chemotherapy stop the production of blood cells in the bone marrow and can be harmful. Stem cells collected from people before high‐dose chemotherapy can be transplanted back to the person if the blood cell count gets too low; this is called autologous hematopoietic stem cell transplantation, or high dose therapy with stem cell rescue. Research to investigate if using high‐dose chemotherapy followed by autologous hematopoietic stem cell transplantation was more favourable than standard‐dose chemotherapy<ref>{{cite journal | vauthors = Peinemann F, Enk H, Smith LA | title = Autologous hematopoietic stem cell transplantation following high-dose chemotherapy for nonrhabdomyosarcoma soft tissue sarcomas | journal = The Cochrane Database of Systematic Reviews | volume = 4 | pages = CD008216 | date = April 2017 | issue = 7 | pmid = 28407197 | pmc = 6478255 | doi = 10.1002/14651858.cd008216.pub5 }}</ref> found only one RCT and this did not favour either of the two treatment arms with respect to overall survival. As a result, high dose chemotherapy with stem cell rescue is generally considered appropriate only in the research setting. == Prognosis == === Factors that affect prognosis === The AJCC has identified several factors that affect prognosis of '''bone sarcomas''':<ref name=":0" /> * '''Size of the tumor''': larger tumors tend to have a worse prognosis compared to smaller tumors. * '''Spread of tumor to surrounding tissues''': tumors that have spread locally to surrounding tissues tend to have a worse prognosis compared to tumors that have not spread beyond their place of origin. * '''Stage and presence of metastases''': tumors that have spread ("metastasized") to the lymph nodes (which is rare for bone sarcomas) or other organs or tissues (for example, to the lungs) have a worse prognosis compared to tumors that have not metastasized. * '''Tumor grade''': higher grade tumors (grades 2 and 3) tend to have a worse prognosis compared to low grade (grade 1) tumors. * '''Skeletal location''': tumors originating in the spine or pelvic bones tend to have a worse prognosis compared to tumors originating in arm or leg bones. For '''soft-tissue sarcomas other than GISTs,''' factors that affect prognosis include:<ref name=":0" /> * '''Stage''': as with bone sarcomas, tumors that have metastasized have a worse prognosis compared to tumors that have not metastasized. * '''Grade''': the AJCC recommends using a grading system called the French Federation of Cancer Centers Sarcoma Group (FNCLCC) Grade for soft-tissue sarcomas, with high-grade tumors having a worse prognosis compared to low-grade tumors. For '''GISTs''', the key factor that affects prognosis is:<ref name=":0" /> * '''Mitotic rate''': [[Mitosis|mitotic]] rate refers to the fraction of [[Cell (biology)|cells]] that are actively [[Cell division|dividing]] within the tumor; GISTs that have a high mitotic rate have a worse prognosis compared to GISTs that have a low mitotic rate. === Outcome data === According to data published by the US National Cancer Institute (NCI), the overall 5-year survival for '''bone sarcomas''' is 66.9%.<ref name=":8">{{Cite web|url=https://seer.cancer.gov/statfacts/html/bones.html|title=Bone and Joint Cancer - Cancer Stat Facts|website=SEER|language=en|access-date=2019-03-27}}</ref> The American Cancer Society (ACS) estimates that 2,140 people in the US will die in 2023 from bone sarcomas, accounting for 0.3% of all cancer deaths.<ref name=":7">{{Cite journal |last1=Siegel |first1=Rebecca L. |last2=Miller |first2=Kimberly D. |last3=Wagle |first3=Nikita Sandeep |last4=Jemal |first4=Ahmedin |date=2023 |title=Cancer statistics, 2023 |journal=CA: A Cancer Journal for Clinicians |volume=73 |issue=1 |pages=17–48 |doi=10.3322/caac.21763 |issn=1542-4863 |pmid=36633525|doi-access=free }}</ref> The [[median]] age at death is 61 years old, although death can occur in any age group.<ref name=":8" /> Thus, 12.3% of bone sarcoma deaths occur in people under 20 years old, 13.8% occur in people 20–34 years old, 5.5% occur in people 35–44 years old, 9.3% occur in people 45–54 years old, 13.5% occur in people 55–64 years old, 16.2% occur in people 65–74 years old, 16.4% occur in people 75–84 years old, and 13.1% occur in people 85 years or older.<ref name=":8" /> For '''soft-tissue sarcomas''', the overall 5-year survival (irrespective of stage) is 64.5%, but survival is affected by many factors, including stage.<ref name=":9">{{Cite web|url=https://seer.cancer.gov/statfacts/html/soft.html|title=Soft Tissue Cancer - Cancer Stat Facts|website=SEER|language=en|access-date=2019-03-27}}</ref> Thus, the 5-year survival is 80.8% for soft-tissue sarcomas that have not spread beyond the primary tumor ("localized" tumors), 58.0% for soft-tissue sarcomas that have spread only to nearby lymph nodes, and 16.4% for soft-tissue sarcomas that have spread to distant organs.<ref name=":9" /> The ACS estimates that 5,140 people will die from soft-tissue sarcoma in 2023, accounting for 0.9% of all cancer deaths.<ref name=":7" /> ==Epidemiology== Sarcomas are rare cancers.<ref name=":1" /> The risk of a previously healthy person receiving a new diagnosis of bone cancer is less than 0.001%, while the risk of receiving a new diagnosis of soft-tissue sarcoma is between 0.0014 and 0.005%.<ref name=":2" /> The American Cancer Society estimates that in the United States there will be 3,970 new cases of bone sarcoma in 2023, and 13,400 new cases of soft-tissue sarcoma.<ref name=":7" /> Considering that the total estimated number of new cancer diagnoses (all types of cancer) is 1,958,310, this means bone sarcomas represent only 0.2% of all new cancer diagnoses (making them the 30th most common type of cancer<ref name=":8" />) and soft-tissue sarcomas represent only 0.7% (making them the 22nd most common type of cancer<ref name=":9" />) of all new cancer diagnoses in the US in 2023.<ref name=":7" /> These estimates are similar to previously reported data.<ref name=":2" /> Sarcomas affect people of all ages. Around 50% of bone sarcomas and 20% of soft-tissue sarcomas are diagnosed in people under the age of 35.<ref>{{cite journal |vauthors=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 |access-date=2012-10-06 |archive-date=25 July 2013 |archive-url=https://web.archive.org/web/20130725060105/http://sarcomahelp.org/articles/sarcoma-statistics.html |url-status=dead }}</ref> Some sarcomas, such as [[leiomyosarcoma]], [[chondrosarcoma]], and [[gastrointestinal stromal tumor]] (GIST), are more common in adults than in children.<ref name=":1" /> Most high-grade bone sarcomas, including [[Ewing's sarcoma|Ewing sarcoma]] and [[osteosarcoma]], are much more common in children and young adults.<ref name=":1" /> == In fossils == In 2016, scientists reported the discovery of an [[osteosarcoma]] tumor in a 1.6–1.8 million-year-old fossil from the skeleton of the now-extinct [[hominin]] species ''[[Australopithecus sediba]]'', making it the earliest-known case of human cancer.<ref>{{Cite web|url=https://www.cnn.com/2016/07/28/health/oldest-human-cancer-found/index.html|title=Scientists find cancer in million-year-old fossil| vauthors = Willingham AJ |website=CNN|date=28 July 2016|access-date=2019-03-27}}</ref><ref>{{cite journal | vauthors = Randolph-Quinney PS, Williams SA, Steyn M, Meyer MR, Smilg JS, Churchill SE, Odes EJ, Augustine T, Tafforeau P, Berger LR |title=Osteogenic tumour in Australopithecus sediba: Earliest hominin evidence for neoplastic disease |journal=South African Journal of Science |date=2016 |volume=112 |issue=7/8 | page = 7 | doi = 10.17159/sajs.2016/20150470 |url=https://sajs.co.za/article/view/3562|doi-access=free }}</ref> == Research == Treatment of sarcoma, especially when the sarcoma has spread, or "metastasized", often requires chemotherapy but existing chemotherapeutic medicines are associated with significant toxicities and are not highly effective in killing cancer cells.<ref name=":2" /> Therefore, research to identify new medications to treat sarcoma is being conducted {{as of|2023|alt=as of 2019}}.<ref name=":2" /> One new type of therapy still under investigation is the use of [[cancer immunotherapy]] (e.g., immune checkpoint inhibitors like anti-PD1, anti-PDL1, and anti-CTLA4 agents) to treat sarcomas.<ref name=":10">{{cite journal | vauthors = Thanindratarn P, Dean DC, Nelson SD, Hornicek FJ, Duan Z | title = Advances in immune checkpoint inhibitors for bone sarcoma therapy | journal = Journal of Bone Oncology | volume = 15 | pages = 100221 | date = April 2019 | pmid = 30775238 | pmc = 6365405 | doi = 10.1016/j.jbo.2019.100221 | doi-access = free }}</ref> These drugs are not yet FDA- or other regulator-approved treatment, except PDL1 inhibitor atezolizumab for the ultra-rate diagnosis of alveolar soft part sarcoma.<ref name=":10" /><ref>{{Cite journal |last1=Chen |first1=Alice P. |last2=Sharon |first2=Elad |last3=O'Sullivan-Coyne |first3=Geraldine |last4=Moore |first4=Nancy |last5=Foster |first5=Jared C. |last6=Hu |first6=James S. |last7=Van Tine |first7=Brian A. |last8=Conley |first8=Anthony P. |last9=Read |first9=William L. |last10=Riedel |first10=Richard F. |last11=Burgess |first11=Melissa A. |last12=Glod |first12=John |last13=Davis |first13=Elizabeth J. |last14=Merriam |first14=Priscilla |last15=Naqash |first15=Abdul R. |date=2023-09-07 |title=Atezolizumab for Advanced Alveolar Soft Part Sarcoma |journal=The New England Journal of Medicine |volume=389 |issue=10 |pages=911–921 |doi=10.1056/NEJMoa2303383 |issn=1533-4406 |pmid=37672694|pmc=10729808 }}</ref> Other strategies, such as small-molecule [[targeted therapy]], biologic agents (e.g., [[small interfering RNA]] molecules), and [[nanoparticle]]-directed therapy, also are under active investigated.<ref name=":2" /> Research to understand the specific genetic and molecular factors that cause sarcoma to develop is underway.<ref name=":2" /> This could allow for the design of new targeted therapies and allow physicians to more accurately predict a patient's prognosis.<ref name=":2" /> ==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|date=19 December 2013 }}</ref> The UK has a Sarcoma Awareness Week in July 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 |access-date=13 April 2018 |date=25 January 2016 |archive-date=22 June 2018 |archive-url=https://web.archive.org/web/20180622033329/https://sarcoma.org.uk/get-involved/SAW |url-status=dead }}</ref> American YouTuber [[Technoblade]] was diagnosed with sarcoma in August 2021, and died from his illness in June 2022 after the sarcoma metastasized. He had raised over $500,000 in a charity stream. Many YouTubers have raised awareness and donated to charities such as the [[Sarcoma Foundation of America]] after Technoblade's diagnosis and passing. To date, Technoblade's fans have raised over $1,000,000 for sarcoma research.<ref>{{Cite web |last=Saunders |first=Cindy |date=2022-07-01 |title=Technoblade Tribute |url=https://www.curesarcoma.org/technoblade-tribute/ |access-date=2022-11-10 |website=Sarcoma Foundation of America |language=en-US}}</ref> [[TikTok]] has provided a voice for many creators to chronicle their experiences with sarcoma. "Dance You Outta My Head", by American singer [[Cat Janice]] went viral on TikTok in early 2024 before the singer died of sarcoma, prompting awareness of this rare disease.<ref>{{Citation |title=Cat Janice |date=2024-05-15 |work=Wikipedia |url=https://en.wikipedia.org/enwiki/w/index.php?title=Cat_Janice&oldid=1223929472 |access-date=2024-05-17 |language=en}}</ref> Kimberley Nix, a Canadian physician, chronicled her journey with undifferentiated pleomorphic sarcoma, from her diagnosis to eventual death, on TikTok under the username @cancerpatientmd.<ref>{{Cite web |last=Archive |first=View Author |last2=feed |first2=Get author RSS |date=2024-05-10 |title=TikTok star Kimberley Nix announces own death in farewell video |url=https://nypost.com/2024/05/10/lifestyle/tiktok-star-kimberley-nix-announces-own-death-in-farewell-video/ |access-date=2024-05-17 |language=en-US}}</ref> Nix died on May 8, 2024 at the age of 31, and her death was announced in a video uploaded posthumously to her TikTok page.<ref>{{Cite web |title=Obituary of Kimberley Marie Nix {{!}} McInnis & Holloway, Park Memorial |url=https://mhfh.com/tribute/details/49347/Kimberley-Nix/obituary.html |access-date=2024-05-17 |website=mhfh.com |language=en-US}}</ref> In many of her videos, she links viewers to her Own.Cancer fundraiser, which has raised almost $118 000 CAD as of May 17, 2024.<ref>{{Cite web |title=OWNP2P |url=https://donate.owncancer.ca/fundraiser/5510855 |access-date=2024-05-17 |website=donate.owncancer.ca}}</ref> == References == {{Reflist}} == External links == * [http://www.cancer.gov/types/bone/hp Bone sarcoma] at the National Cancer Institute * [https://www.reininsarcoma.org/what-is-sarcoma-2/ What is Sarcoma?] {{Webarchive|url=https://web.archive.org/web/20210805153821/https://www.reininsarcoma.org/what-is-sarcoma-2/ |date=5 August 2021 }} * [https://sarcomahelp.org/ Sarcoma Help from the Liddy Shriver Sarcoma Initiative] {{Medical resources | DiseasesDB = | ICD10 = | ICD9 = | ICDO = {{ICDO|8800|3}} | OMIM = | MedlinePlus = | eMedicineSubj = | eMedicineTopic = | MeshID = D012509 | SNOMED CT = 424413001 }} {{Soft tissue tumors and sarcomas |state=collapsed}} {{Osseous and chondromatous tumors |state=collapsed}} {{Vascular tumors |state=collapsed}} {{Authority control}} [[Category:Sarcoma| ]] [[Category:Anatomical pathology]] [[Category:Soft tissue disorders]]'