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==Overview==
==Overview==
A sarcoma (from the Greek 'sarx' meaning "flesh") is a [[cancer]] of the [[connective tissue|connective or supportive tissue]] ([[bone]], [[cartilage]], [[fat]], [[muscle]], [[blood vessel]]s) and [[soft tissue]].  This is in contrast to [[Carcinoma|carcinomas]], which are of [[Epithelium|epithelial]] origin ([[breast]], [[Colon (anatomy)|colon]], [[pancreas]], and others).   
A sarcoma (from the Greek 'sarx' meaning "[[flesh]]") is a [[cancer]] of the [[connective tissue|connective or supportive tissue]] ([[bone]], [[cartilage]], [[fat]], [[muscle]], [[blood vessel]]s) and [[soft tissue]].  This is in contrast to [[Carcinoma|carcinomas]], which are of [[Epithelium|epithelial]] origin ([[breast]], [[Colon (anatomy)|colon]], [[pancreas]], and others). It can be [[Classification|classified]] based on the [[Tissue (biology)|tissue]] involved and the [[histology]] of the [[lesion]]. [[Soft tissue]] sarcoma needs to differentiated from [[soft tissue]] [[Benign tumor|benign tumors]] such as [[Adenoma]], [[lipoma]], and [[fibroma]]. The estimated [[incidence]] of [[soft tissue]] sarcoma worldwide is 1.8 to 5 per 100,000 per year. [[Soft tissue]] sarcomas are more commonly found in older [[Patient|patients]] (>50 years old). [[Risk factor|Risk factors]] include [[Radiation therapy|radiation]] exposure, damaged [[lymphatic system]], and [[inherited]] conditions. There is insufficient evidence to recommend routine [[Screening (medicine)|screening]] for sarcoma. If left untreated, sarcoma can lead to [[Complication (medicine)|complications]] of local [[Tissue (biology)|tissue]] erosion, compression and [[Invasive (medical)|invasion]]. [[Complication (medicine)|Complications]] can also include [[Adverse effect (medicine)|side effects]] of [[chemotherapy]] and [[radiation therapy]]. [[Prognosis]] depends upon the size and [[Cancer staging|stage]] of the [[tumor]], age and general health of the [[patient]], and [[benign]]/[[malignant]] nature of the [[tumor]]. Sarcoma can be [[Diagnosis|diagnosed]] by combination of [[imaging]] and [[biopsy]]. Symptoms include painless [[swelling]] or [[lump]], [[Menstrual disorder|menstrual irregularities]], [[constipation]], and [[indigestion]]. [[Chemotherapy]] may be used with [[radiation therapy]] either before or after [[surgery]] to try to shrink the [[tumor]] or kill any remaining [[cancer]] [[cells]]. [[Surgery]] is the most common treatment for [[soft tissue]] sarcomas. It is important to obtain a margin free of [[tumor]] to decrease the likelihood of local recurrence and give the best chance for eradication of the [[tumor]]. [[Radiation therapy]] (treatment with [[x-rays]] or [[radioactive]] implants) may be used either before [[surgery]] to shrink [[tumors]] or after [[surgery]] to kill any [[cancer]] [[Cell (biology)|cells]] that may have been left behind. There are no established measures for the [[Prevention (medical)|primary and secondary prevention]] of sarcoma.   


==Classification==
==Classification==
Sarcomas are given a number of different names, based on the type of tissue from which they arise.  For example, [[osteosarcoma]] arises from [[bone]], [[chondrosarcoma]] arises from [[cartilage]], and [[leiomyosarcoma]] arises from [[smooth muscle]].  Sarcomas strike people in all age ranges, but they are very rare, accounting for only 1% of all cases of [[cancer]].<ref>Borden EC, Baker LH, Bell RS, Bramwell V, Demetri GD, Eisenberg BL, Fletcher CD, Fletcher JA, Ladanyi M, Meltzer P, O'Sullivan B, Parkinson DR, Pisters PW, Saxman S, Singer S, Sundaram M, van Oosterom AT, Verweij J, Waalen J, Weiss SW, Brennan MF. Soft tissue sarcomas of adults: state of the translational science.Clin Cancer Res. 2003 Jun;9(6):1941-56. Review. PMID 12796356</ref>  Soft tissue sarcomas, such as [[leiomyosarcoma]], [[chondrosarcoma]], and [[gastrointestinal stromal tumor]] (GIST), are more common in adults than in children.  GIST is the most common form of sarcoma, with approximately 3000-3500 cases per year in the United States.<ref>Tran T, Davila JA, El-Serag HB. The epidemiology of malignant gastrointestinal stromal tumors: an analysis of 1,458 cases from 1992 to 2000. Am J Gastroenterol 2005;100:162-8. PMID 15654796 </ref> This should be compared with breast cancer, with approximately 200,000 cases per year in North America.<ref>Smigal C, Jemal A, Ward E, Cokkinides V, Smith R, Howe HL, Thun M. Trends in breast cancer by race and ethnicity: update 2006. CA Cancer J Clin 2006;56:168-83. PMID 16737949 </ref> Bone sarcomas, such as [[osteosarcoma]] and [[Ewing's sarcoma]], are more common in children than in adults.  These tumors most commonly strike adolescents and young adults between the ages of 12 and 25. In addition to being named based on the tissue of origin, sarcomas are also assigned a grade, such as low grade or high grade.  Low grade sarcomas are usually treated surgically, although sometimes [[radiation therapy]] or [[chemotherapy]] are used.  High grade sarcomas are more frequently treated with [[chemotherapy]].  Since these tumors are more likely to undergo [[metastasis]] (spreading to distant sites), these tumors are treated more aggressively.  Childhood sarcomas are almost always treated with a combination of [[surgery]] and [[chemotherapy]], and radiation is frequently used as well.  The recognition that childhood 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>Longhi A, Errani C, De Paolis M, Mercuri M, Bacci G. Primary bone osteosarcoma in the pediatric age: state of the art.  Cancer Treat Rev. 2006;32:423-36. PMID 16860938 </ref>
* Sarcomas are given a number of different names, based on the type of [[Tissue (biology)|tissue]] from which they arise.  For example, [[osteosarcoma]] arises from [[bone]], [[chondrosarcoma]] arises from [[cartilage]], and [[leiomyosarcoma]] arises from [[smooth muscle]].   
* Sarcomas strike people in all age ranges, but they are very rare, accounting for only 1% of all cases of [[cancer]].<ref>Borden EC, Baker LH, Bell RS, Bramwell V, Demetri GD, Eisenberg BL, Fletcher CD, Fletcher JA, Ladanyi M, Meltzer P, O'Sullivan B, Parkinson DR, Pisters PW, Saxman S, Singer S, Sundaram M, van Oosterom AT, Verweij J, Waalen J, Weiss SW, Brennan MF. Soft tissue sarcomas of adults: state of the translational science.Clin Cancer Res. 2003 Jun;9(6):1941-56. Review. PMID 12796356</ref>   
* Soft tissue sarcomas, such as [[leiomyosarcoma]], [[chondrosarcoma]], and [[gastrointestinal stromal tumor|gastrointestinal stromal tumor (GIST)]], are more common in [[Adult|adults]] than in children.   
* [[Gastrointestinal stromal tumor|GIST]] is the most common form of sarcoma, with approximately 3000 - 3500 cases per year in the United States.<ref>Tran T, Davila JA, El-Serag HB. The epidemiology of malignant gastrointestinal stromal tumors: an analysis of 1,458 cases from 1992 to 2000. Am J Gastroenterol 2005;100:162-8. PMID 15654796 </ref>
* [[Osteosarcoma|Bone sarcomas]], such as [[osteosarcoma]] and [[Ewing's sarcoma]], are more common in children than in [[Adult|adults]].  These tumors most commonly strike adolescents and young adults between the ages of 12 and 25.  
* In addition to being named based on the [[Tissue (biology)|tissue]] of origin, sarcomas are also assigned a grade, such as low grade or high grade.   
* Low grade sarcomas are usually treated [[Surgery|surgically]], although sometimes [[radiation therapy]] or [[chemotherapy]] are used.   
* High grade sarcomas are more frequently treated with [[chemotherapy]].  Since these [[Tumor|tumors]] are more likely to undergo [[metastasis]] (spreading to distant sites), these [[Tumor|tumors]] are treated more aggressively.   
* Childhood sarcomas are almost always treated with a combination of [[surgery]] and [[chemotherapy]], and [[radiation]] is frequently used as well.   
* The recognition that childhood sarcomas are sensitive to [[chemotherapy]] has dramatically improved the survival of [[Patient|patients]].  For example, in the era before [[chemotherapy]], long term survival for [[Patient|patients]] with localized [[osteosarcoma]] was only approximately 20%, but now, it has risen to 60 - 70%.<ref>Longhi A, Errani C, De Paolis M, Mercuri M, Bacci G. Primary bone osteosarcoma in the pediatric age: state of the art.  Cancer Treat Rev. 2006;32:423-36. PMID 16860938 </ref>
 
===Tables ===
===Tables ===


{| class="wikitable"
{| class="wikitable"
|+ Table 1: Major Types of Soft-Tissue Sarcomas in Adults
|+ Table 1: Major Types of Soft-Tissue Sarcomas in Adults
|- valign=top
|- valign="top"
! <u>Tissue of Origin</u>
! <u>Tissue of Origin</u>
! <u>Type of Cancer</u>
! <u>Type of Cancer</u>
! <u>Usual Location in the Body</u>
! <u>Usual Location in the Body</u>
|- valign=top
|- valign="top"
| '''Fibrous tissue'''
| '''Fibrous tissue'''
| [[Fibrosarcoma]]
| [[Fibrosarcoma]]
| Arms, legs, trunk
| [[Arm|Arms]], [[Leg|legs]], [[trunk]]
|- valign="TOP"
|- valign="TOP"
|
|
| [[Malignant fibrous histiocytoma|Malignant fibrous<br>hystiocytoma]] || Legs
| [[Malignant fibrous histiocytoma|Malignant fibrous<br>hystiocytoma]] || [[Leg|Legs]]
|- valign="TOP"
|- valign="TOP"
|
|
| [[Dermatofibrosarcoma]] || Trunk
| [[Dermatofibrosarcoma]] || [[Trunk]]
|- valign=top
|- valign="top"
| '''Fat'''
| '''Fat'''
| [[Liposarcoma]]
| [[Liposarcoma]]
| Arms, legs, trunk
| [[Arm|Arms]], [[Leg|legs]], [[trunk]]
|- valign=top
|- valign="top"
| '''Muscle'''<br><dd>Striated muscle<dd>Smooth muscle
| '''Muscle'''<br><dd>[[Striated muscle]]<dd>[[Smooth muscle]]
| <br>[[Rhabdomyosarcoma]]<br />[[Leiomyosarcoma]]
| <br>[[Rhabdomyosarcoma]]<br />[[Leiomyosarcoma]]
| valign=bottom | <br>Arms, legs<br />Uterus, digestive tract
| valign="bottom" | <br>[[Arm|Arms]], [[Leg|legs]]<br />[[uterus]], [[Gastrointestinal tract|digestive tract]]
|- valign=top
|- valign="top"
| '''Blood vessels'''
| '''Blood vessels'''
| [[Hemangiosarcoma]]
| [[Hemangiosarcoma]]
| Arms, legs, trunk
| [[Arm|Arms]], [[Leg|legs]], [[trunk]]
|- valign="TOP"
|- valign="TOP"
| || [[Kaposi's sarcoma]] || Legs, trunk
| || [[Kaposi's sarcoma]] || [[Leg|Legs]], [[trunk]]
|- valign=top
|- valign="top"
| '''Lymph vessels'''
| '''Lymph vessels'''
| [[Lymphangiosarcoma]]
| [[Lymphangiosarcoma]]
| Arms
| [[Arm|Arms]]
|- valign=top
|- valign="top"
| '''Synovial tissue'''<br>(linings of joint cavities, tendon sheaths)
| '''Synovial tissue'''<br>(linings of [[joint]] cavities, [[Tendon sheath|tendon sheaths]])
| [[Synovial sarcoma]]
| [[Synovial sarcoma]]
| Legs
| [[Leg|Legs]]
|- valign=top
|- valign="top"
| '''Peripheral nerves'''
| '''Peripheral nerves'''
| [[Malignant peripheral nerve sheath tumour]]/[[Neurofibrosarcoma]]
| [[Malignant]] [[Peripheral nervous system|peripheral nerve]] sheath [[tumor]]/[[Neurofibrosarcoma]]
| Arms, legs, trunk
| [[Arm|Arms]], [[Leg|legs]], [[trunk]]
|- valign=top
|- valign="top"
| '''Cartilage and bone-forming tissue'''
| '''Cartilage and bone-forming tissue'''
| [[Extraskeletal chondrosarcoma]]
| Extraskeletal [[chondrosarcoma]]
| Legs
| [[Leg|Legs]]
|- valign="TOP"
|- valign="TOP"
|
|
| [[Extraskeletal osteosarcoma]] || Legs, trunk (not involving the bone)
| [[Extraskeletal osteosarcoma]] || [[Leg|Legs]], [[trunk]] (not involving the [[bone]])
|}
|}
{| class="wikitable"
{| class="wikitable"
|+ Table 2: Major Types of Soft-Tissue Sarcomas in Children
|+ Table 2: Major Types of Soft-Tissue Sarcomas in Children
|- valign=top
|- valign="top"
! <u>Tissue of Origin</u>
! <u>Tissue of Origin</u>
! <u>Type of Cancer</u>
! <u>Type of Cancer</u>
! <u>Usual Location in the Body</u>
! <u>Usual Location in the Body</u>
! <u>Most common ages</u>
! <u>Most common ages</u>
|- valign=top
|- valign="top"
| '''Muscle'''
| '''Muscle'''
|
|
|  
|  
|
|
|- valign=top
|- valign="top"
| <dd>Striated<br><dd>muscle
| <dd>[[Striated muscle|Striated<br>]]<dd>[[Striated muscle|muscle]]
| Rhabdomyosarcoma
| [[Rhabdomyosarcoma]]
|
|
|
|
|- valign=top
|- valign="top"
|
|
| <dd>Embryonal
| <dd>[[Germ cell tumor|Embryonal]]
| Head and neck, genitourinary tract
| [[Head]] and [[neck]], [[genitourinary tract]]
| Infant–4
| Infant–4
|- valign=top
|- valign="top"
|
|
| <dd>[[Alveolar soft part sarcoma]]
| <dd>[[Alveolar soft part sarcoma]]
| Arms, legs, head, and neck
| [[Arm|Arms]], [[Leg|legs]], [[head]], and [[neck]]
| Infant–19
| Infant–19
|- valign=top
|- valign="top"
| <dd>Smooth<br><dd>muscle
| <dd>[[Smooth muscle|Smooth<br>]]<dd>[[Smooth muscle|muscle]]
| Leiomyosarcoma
| [[Leiomyosarcoma]]
| Trunk
| [[Trunk]]
| 15–19
| 15–19
|- valign=top
|- valign="top"
| '''Fibrous tissue'''
| '''Fibrous tissue'''
| Fibrosarcoma
| [[Fibrosarcoma]]
| Arms and legs
| [[Arm|Arms]] and [[Leg|legs]]
| 15–19
| 15–19
|- valign=top
|- valign="top"
|
|
|  Malignant fibrous<br>histiocytoma
|  [[Malignant fibrous histiocytoma|Malignant fibrous<br>histiocytoma]]
| Legs
| [[Leg|Legs]]
| 15–19
| 15–19
|- valign=top
|- valign="top"
|
|
|  Dermatofibrosarcoma
|  [[Dermatofibrosarcoma]]
| Trunk
| [[Trunk]]
| 15–19
| 15–19
|- valign=top
|- valign="top"
| '''Fat'''
| '''Fat'''
| Liposarcoma
| [[Liposarcoma]]
| Arms and Legs
| [[Arm|Arms]] and [[Leg|Legs]]
| 15–19
| 15–19
|- valign=top
|- valign="top"
| '''Blood vessels'''
| '''Blood vessels'''
| Infantile hemangio-<br><dd>pericytoma
| Infantile hemangio-<br><dd>pericytoma
| Arms, legs, trunk, head, and neck
| [[Arm|Arms]], [[Leg|legs]], [[trunk]], [[head]], and [[neck]]
| Infant–4
| Infant–4
|- valign=top
|- valign="top"
| '''Synovial tissue'''<br>(linings of joint cavities, tendon sheaths)
| '''Synovial tissue'''<br>(linings of [[joint]] cavities, [[Tendon sheath|tendon sheaths]])
| Synovial sarcoma
| [[Synovial sarcoma]]
| Legs, arms, and trunk
| [[Leg|Legs]], [[Arm|arms]], and [[trunk]]
| 15–19
| 15–19
|- valign=top
|- valign="top"
| '''Peripheral nerves'''
| '''Peripheral nerves'''
| Malignant peripheral nerve sheath tumors (also called neurofibrosarcomas, malignant schwannomas, and neurogenic sarcomas)
| [[Malignant peripheral nerve sheath tumor]] (also called [[Neurofibrosarcoma|neurofibrosarcomas]], [[malignant]] [[Schwannoma|schwannomas]], and [[neurogenic]] sarcomas)
| Arms, legs, and trunk
| [[Arm|Arms]], [[Leg|legs]], and [[trunk]]
| 15–19
| 15–19
|- valign=top
|- valign="top"
| '''Muscular nerves'''
| '''Muscular nerves'''
| [[Alveolar soft part sarcoma]]
| [[Alveolar soft part sarcoma]]
| Arms and legs
| [[Arm|Arms]] and [[Leg|legs]]
| Infant–19
| Infant–19
|- valign=top
|- valign="top"
| '''Cartilage and bone-forming tissue'''
| '''Cartilage and bone-forming tissue'''
| [[Extraskeletal myxoid chondrosarcoma]]
| Extraskeletal myxoid [[chondrosarcoma]]
| Legs
| [[Leg|Legs]]
| 10–14
| 10–14
|-
|-
|
|
| Extraskeletal mesenchymal
| Extraskeletal [[mesenchymal]]
| Legs
| [[Leg|Legs]]
| 10–14
| 10–14
|}
|}


== Types of sarcoma ==
=== Types of sarcoma ===
([[ICD-O]] codes are provided where available.)
([[ICD-O]] codes are provided where available.)
* Askin's Tumor (8803/3)
* Askin's [[tumor]] (8803/3)
* [[Chondrosarcoma]] (9220/3-9240/3)
* [[Chondrosarcoma]] (9220/3-9240/3)
* [[Ewing's]] (9260/3) - [[Primitive neuroectodermal tumor|PNET]] (9473/3)
* [[Ewing's]] (9260/3) - [[Primitive neuroectodermal tumor|PNET]] (9473/3)
Line 160: Line 170:
**[[Desmoplastic small round cell tumor]] (8806/3)
**[[Desmoplastic small round cell tumor]] (8806/3)
**[[Epithelioid Sarcoma]] (8804/3)
**[[Epithelioid Sarcoma]] (8804/3)
**Extraskeletal chondrosarcoma (9220/3)
**Extraskeletal [[chondrosarcoma]] (9220/3)
**Extraskeletal osteosarcoma (9180/3)
**Extraskeletal [[osteosarcoma]] (9180/3)
**[[Fibrosarcoma]] (8810/3)
**[[Fibrosarcoma]] (8810/3)
**[[Hemangiopericytoma]] (9150)
**[[Hemangiopericytoma]] (9150)
Line 169: Line 179:
**[[Liposarcoma]] (8850/3-8858/3)
**[[Liposarcoma]] (8850/3-8858/3)
**[[Lymphangiosarcoma]] (9170-9175)
**[[Lymphangiosarcoma]] (9170-9175)
**[[Lymphoma|Lymphosarcoma]]
**[[Lymphosarcoma]]
**[[Malignant fibrous histiocytoma]] (8830/3)
**[[Malignant fibrous histiocytoma]] (8830/3)
**[[Neurofibrosarcoma]] (9540/3)
**[[Neurofibrosarcoma]] (9540/3)
**[[Rhabdomyosarcoma]] (8900-8920)
**[[Rhabdomyosarcoma]] (8900-8920)
**[[Synovial sarcoma]] (9040/3-9043/3)
**[[Synovial sarcoma]] (9040/3-9043/3)
 
==Differentiating Sarcoma from Other Diseases==
==Causes==
[[Soft tissue]] sarcoma needs to differentiated from [[soft tissue]] [[Benign tumor|benign tumors]] such as:
==Differential Diagnosis==
Soft tissue sarcoma needs to differentiate from soft tissue benige tumors such as:


*[[Adenoma]]
*[[Adenoma]]
Line 184: Line 192:


*[[Fibroma]]
*[[Fibroma]]
 
==Epidemiology and Demographics==
===Drug Side Effect===
 
* [[Cyclophosphamide]]
==Epidemiology==
===Incidence===
===Incidence===
* The estimated number of new cases of soft tissue sarcoma in the United States is approximately 12,000.<ref name=ACS>American Cancer Society: Cancer Facts and Figures 2014. Atlanta, Ga: American Cancer Society, 2014. [http://www.cancer.org/acs/groups/content/@research/documents/webcontent/acspc-042151.pdf Available online] . Last accessed May 21, 2014.</ref>
* The estimated number of new cases of [[soft tissue]] sarcoma in the United States is approximately 12,000.<ref name="ACS">American Cancer Society: Cancer Facts and Figures 2014. Atlanta, Ga: American Cancer Society, 2014. [http://www.cancer.org/acs/groups/content/@research/documents/webcontent/acspc-042151.pdf Available online] . Last accessed May 21, 2014.</ref>


* The estimated incidence of soft tissue sarcoma worldwide is 1.8 to 5 per 100,000 per year.<ref name="pmid19858086">{{cite journal| author=Wibmer C, Leithner A, Zielonke N, Sperl M, Windhager R| title=Increasing incidence rates of soft tissue sarcomas? A population-based epidemiologic study and literature review. | journal=Ann Oncol | year= 2010 | volume= 21 | issue= 5 | pages= 1106-11 | pmid=19858086 | doi=10.1093/annonc/mdp415 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19858086  }} </ref>
* The estimated [[incidence]] of [[soft tissue]] sarcoma worldwide is 1.8 to 5 per 100,000 per year.<ref name="pmid19858086">{{cite journal| author=Wibmer C, Leithner A, Zielonke N, Sperl M, Windhager R| title=Increasing incidence rates of soft tissue sarcomas? A population-based epidemiologic study and literature review. | journal=Ann Oncol | year= 2010 | volume= 21 | issue= 5 | pages= 1106-11 | pmid=19858086 | doi=10.1093/annonc/mdp415 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19858086  }} </ref>


===Age===
===Age===
Soft tissue sarcomas are more commonly found in older patients (>50 years old) although in children and adolescents under age 20, certain histologies are common (rhabdomyosarcoma).
* [[Soft tissue]] sarcomas are more commonly found in older [[Patient|patients]] (>50 years old).
* Certain [[Histology|histological]] sub-types are more common in children and [[Adolescence|adolescents]] under age 20 ([[rhabdomyosarcoma]]).


===Percent Distribution of Soft Tissue Sarcoma by Histology===
===Percent Distribution of Soft Tissue Sarcoma by Histology===
* Fibrosarcoma: 6.9%
* [[Fibrosarcoma]]: 6.9%
* Infantile fibrosarcoma: 0.2%
* [[Infant|Infantile]] [[fibrosarcoma]]: 0.2%
* Fibrous histiocytoma, malignant: 9.2%
* [[Fibrous histiocytoma]], [[malignant]]: 9.2%
* Dermatofibrosarcoma: 3.6%
* [[Dermatofibrosarcoma]]: 3.6%
* Liposarcoma: 17.1%
* [[Liposarcoma]]: 17.1%
* Leiomyosarcoma: 13.2%
* [[Leiomyosarcoma]]: 13.2%
* Rhabdomyosarcoma: 3.1%
* [[Rhabdomyosarcoma]]: 3.1%
* Embryonal rhabdomyosarcoma: 1.3%
* [[Rhabdomyosarcoma|Embryonal rhabdomyosarcoma]]: 1.3%
* Hemangiosarcoma: 3.7%
* [[Hemangiosarcoma]]: 3.7%
* Hemangiopericytoma, malignant: 0.5%
* [[Hemangiopericytoma]], [[malignant]]: 0.5%
* Kaposi sarcoma: 0.8%
* [[Kaposi's sarcoma]]: 0.8%
* Malignant peripheral nerve sheath tumor: 1.6%
* [[Malignant peripheral nerve sheath tumor]]: 1.6%
* Malignant neurilemmoma: 0.2%
* [[Malignant]] [[neurilemmoma]]: 0.2%
* Neuroblastoma: 0.6%
* [[Neuroblastoma]]: 0.6%
* Synovial sarcoma: 4.8%
* [[Synovial sarcoma]]: 4.8%


==Risk Factor==
==Risk Factors==
*Radiation exposure: Clinical stidies suggest that patients with other kind of cancers such as lymphoma and breast cancer may develop sarcomas from radiation therapy. The sarcoma often happens in the area of the body that had been treated with radiation.
*[[Radiation therapy|Radiation]] exposure: [[Clinical trial|Clinical studies]] suggest that [[Patient|patients]] with other kind of [[cancers]] such as [[lymphoma]] and [[breast cancer]] may develop sarcomas from [[radiation therapy]]. The sarcoma often develops in the area of the [[body]] that had been treated with [[Radiation therapy|radiation]].


*Damaged lymph system: Clinical observations demonstrate lymphangiosarcoma is a very rare complication of chronic lymphedema that is the result of damaged lymph system.
*Damaged [[lymphatic system]]: Clinical observations demonstrate that [[lymphangiosarcoma]] is a very rare [[Complication (medicine)|complication]] of [[Chronic (medical)|chronic]] [[lymphedema]] that is the result of damaged [[lymphatic system]].


*Inherited conditions: Some inherited conditions may increase the risk of developing soft tissue sarcomas, such as [[neurofibromatosis]], [[Gardner syndrome]], [[Li-Fraumeni syndrome]], [[Retinoblastoma]],[[Werner syndrome]],
*[[Inherited]] conditions: Some [[inherited]] conditions may increase the risk of developing [[soft tissue]] sarcomas, such as [[neurofibromatosis]], [[Gardner syndrome]], [[Li-Fraumeni syndrome]], [[Retinoblastoma]], and [[Werner syndrome]].


== Screening ==
* There is insufficient evidence to recommend routine [[Screening (medicine)|screening]] for sarcoma.
==Natural History, Complications, and Prognosis==
* If left untreated, sarcoma can lead to [[Complication (medicine)|complications]] of local [[Tissue (biology)|tissue]] erosion, compression and [[Invasive (medical)|invasion]].
* It can also lead to [[metastasis]] to distant sites.
* [[Complication (medicine)|Complications]] can also include [[Adverse effect (medicine)|side effects]] of [[chemotherapy]] and [[radiation therapy]].
* [[Surgery|Surgical]] [[wound]] after [[resection]] can also [[Complication (medicine)|complicate]] a sarcoma.
* The [[prognosis]] of [[soft tissue]] sarcoma is poor and it depends on the following:
*Whether or not the [[tumor]] can be removed by [[surgery]]
*The [[Cancer staging|stage]] of the sarcoma:
** The size of the [[tumor]].
** [[Benign]] or [[malignant]] nature.
*The [[Patient|patient’s]] general [[health]].
*Whether the sarcoma has just been [[Diagnosis|diagnosed]] or has recurred.
==Diagnosis==
=== Diagnostic Study of Choice ===
* Sarcoma can be [[Diagnosis|diagnosed]] by combination of [[imaging]] and [[biopsy]].
===History and Symptoms===
* Painless [[lump]] or [[swelling]]
* [[Pain]] or [[Sore|soreness]]
* [[Dysmenorrhea|Menstrual cramps]]
* [[Indigestion]]
* [[Constipation]]
=== Physical Examination ===
* [[Patient|Patients]] with sarcoma usually appear normal.
* Common [[physical examination]] findings include painless [[lump]] or [[swelling]].
=== Laboratory Findings ===
* There are no [[Diagnosis|diagnostic]] [[Medical laboratory|laboratory]] findings associated with sarcoma.
=== Electrocardiogram ===
* There are no [[The electrocardiogram|ECG]] findings associated with sarcoma.
=== X-ray ===
* [[X-rays|X-ray]] can be the first investigation ordered to evaluate a suspected sarcoma.<ref name=":0">{{Cite web|url=https://www.cancer.org|title=Sarcoma|last=|first=|date=|website=|archive-url=|archive-date=|dead-url=|access-date=}}</ref>
* [[Chest X-ray|Chest x-ray]] can help rule in/out [[metastasis]] to the [[Lung|lungs]].<ref name=":0" />
=== Echocardiography or Ultrasound ===
* [[Ultrasound]] can help determine if a suspected sarcoma is [[fluid]] filled.<ref name=":0" />
* [[Ultrasound]] is usually performed before [[biopsy]].
=== CT scan ===
* [[Computed tomography|CT scan]] can be used as a guiding tool in taking [[biopsy]].<ref name=":0" />
* It can also help in making a [[diagnosis]] of the [[lesion]] itself.
=== MRI ===
* [[Magnetic resonance imaging|MRI]] determines the extent of [[tumor]] [[invasion]].<ref name=":0" />
* It provides a detailed picture of the [[lesion]].<ref name=":0" />
* It can also help in determining the [[Tissue (biology)|tissue]] of origin.
=== Other Imaging Findings ===
* [[Positron emission tomography|PET (positron emission tomography) scan]] can be used to determine the spread of sarcoma.
=== Other Diagnostic Studies ===
* There are no other [[Diagnosis|diagnostic]] studies associated with sarcoma.
==Treatment==
===Medical Therapy===
* [[Chemotherapy]] may be used with [[radiation therapy]] either before or after [[surgery]] to try to shrink the [[tumor]] or kill any remaining [[cancer]] [[cells]].
* In general, the effects of [[chemotherapy]] on [[soft tissue]] sarcoma have had little impact as opposed to other [[Cancer|cancers]]. 
* If the [[cancer]] has spread to other areas of the [[Human body|body]], [[chemotherapy]] may be used to shrink [[tumors]] and reduce the [[pain]] and [[discomfort]].
* The use of [[chemotherapy]] to prevent the spread of [[soft tissue]] sarcomas has not been proven to be effective.
* [[Patient|Patients]] with [[soft tissue]] sarcomas usually receive [[chemotherapy]] [[Intravenous therapy|intravenously]].
===Surgery===
* [[Surgery]] is the most common treatment for [[soft tissue]] sarcomas.
* Depending on the size and location of the sarcoma, it may occasionally be necessary to remove all or part of an [[arm]] or [[leg]] ([[amputation]]).
* In most cases, [[limb]]-sparing [[surgery]] is an option to avoid [[Amputation|amputating]] the [[arm]] or [[leg]].
* It is important to obtain a margin free of [[tumor]] to decrease the likelihood of local recurrence and give the best chance for eradication of the [[tumor]].
===Radiation Therapy===
* [[Radiation therapy]] (treatment with [[x-rays]] or [[radioactive]] implants) may be used either before [[surgery]] to shrink [[tumors]] or after [[surgery]] to kill any [[cancer]] [[Cell (biology)|cells]] that may have been left behind.
* In some cases, it can be used to treat [[tumors]] that cannot be [[Surgery|surgically]] removed. 
* In multiple studies, [[radiation therapy]] has been found to improve the rate of local control, but has not had any influence on overall survival.
=== Primary Prevention ===
* There are no established measures for the [[Prevention (medical)|primary prevention]] of sarcoma.
=== Secondary Prevention ===
* There are no established measures for the [[Prevention (medical)|secondary prevention]] of sarcoma.


==References==
==References==
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Latest revision as of 00:04, 30 July 2020

WikiDoc Resources for Sarcoma

Articles

Most recent articles on Sarcoma

Most cited articles on Sarcoma

Review articles on Sarcoma

Articles on Sarcoma in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Sarcoma

Images of Sarcoma

Photos of Sarcoma

Podcasts & MP3s on Sarcoma

Videos on Sarcoma

Evidence Based Medicine

Cochrane Collaboration on Sarcoma

Bandolier on Sarcoma

TRIP on Sarcoma

Clinical Trials

Ongoing Trials on Sarcoma at Clinical Trials.gov

Trial results on Sarcoma

Clinical Trials on Sarcoma at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Sarcoma

NICE Guidance on Sarcoma

NHS PRODIGY Guidance

FDA on Sarcoma

CDC on Sarcoma

Books

Books on Sarcoma

News

Sarcoma in the news

Be alerted to news on Sarcoma

News trends on Sarcoma

Commentary

Blogs on Sarcoma

Definitions

Definitions of Sarcoma

Patient Resources / Community

Patient resources on Sarcoma

Discussion groups on Sarcoma

Patient Handouts on Sarcoma

Directions to Hospitals Treating Sarcoma

Risk calculators and risk factors for Sarcoma

Healthcare Provider Resources

Symptoms of Sarcoma

Causes & Risk Factors for Sarcoma

Diagnostic studies for Sarcoma

Treatment of Sarcoma

Continuing Medical Education (CME)

CME Programs on Sarcoma

International

Sarcoma en Espanol

Sarcoma en Francais

Business

Sarcoma in the Marketplace

Patents on Sarcoma

Experimental / Informatics

List of terms related to Sarcoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Sabawoon Mirwais, M.B.B.S, M.D.[2]

Overview

A sarcoma (from the Greek 'sarx' meaning "flesh") is a cancer of the connective or supportive tissue (bone, cartilage, fat, muscle, blood vessels) and soft tissue. This is in contrast to carcinomas, which are of epithelial origin (breast, colon, pancreas, and others). It can be classified based on the tissue involved and the histology of the lesion. Soft tissue sarcoma needs to differentiated from soft tissue benign tumors such as Adenoma, lipoma, and fibroma. The estimated incidence of soft tissue sarcoma worldwide is 1.8 to 5 per 100,000 per year. Soft tissue sarcomas are more commonly found in older patients (>50 years old). Risk factors include radiation exposure, damaged lymphatic system, and inherited conditions. There is insufficient evidence to recommend routine screening for sarcoma. If left untreated, sarcoma can lead to complications of local tissue erosion, compression and invasion. Complications can also include side effects of chemotherapy and radiation therapy. Prognosis depends upon the size and stage of the tumor, age and general health of the patient, and benign/malignant nature of the tumor. Sarcoma can be diagnosed by combination of imaging and biopsy. Symptoms include painless swelling or lump, menstrual irregularities, constipation, and indigestion. Chemotherapy may be used with radiation therapy either before or after surgery to try to shrink the tumor or kill any remaining cancer cells. Surgery is the most common treatment for soft tissue sarcomas. It is important to obtain a margin free of tumor to decrease the likelihood of local recurrence and give the best chance for eradication of the tumor. Radiation therapy (treatment with x-rays or radioactive implants) may be used either before surgery to shrink tumors or after surgery to kill any cancer cells that may have been left behind. There are no established measures for the primary and secondary prevention of sarcoma.

Classification

Tables

Table 1: Major Types of Soft-Tissue Sarcomas in Adults
Tissue of Origin Type of Cancer Usual Location in the Body
Fibrous tissue Fibrosarcoma Arms, legs, trunk
Malignant fibrous
hystiocytoma		 Legs
Dermatofibrosarcoma Trunk
Fat Liposarcoma Arms, legs, trunk
Muscle
Striated muscle
Smooth muscle

Rhabdomyosarcoma
Leiomyosarcoma
Arms, legs
uterus, digestive tract
Blood vessels Hemangiosarcoma Arms, legs, trunk
Kaposi's sarcoma Legs, trunk
Lymph vessels Lymphangiosarcoma Arms
Synovial tissue
(linings of joint cavities, tendon sheaths) 		Synovial sarcoma Legs
Peripheral nerves Malignant peripheral nerve sheath tumor/Neurofibrosarcoma Arms, legs, trunk
Cartilage and bone-forming tissue Extraskeletal chondrosarcoma Legs
Extraskeletal osteosarcoma Legs, trunk (not involving the bone)
Table 2: Major Types of Soft-Tissue Sarcomas in Children
Tissue of Origin Type of Cancer Usual Location in the Body Most common ages
Muscle
Striated
muscle
Rhabdomyosarcoma
Embryonal
Head and neck, genitourinary tract Infant–4
Alveolar soft part sarcoma
Arms, legs, head, and neck Infant–19
Smooth
muscle
Leiomyosarcoma Trunk 15–19
Fibrous tissue Fibrosarcoma Arms and legs 15–19
Malignant fibrous
histiocytoma 		Legs 15–19
Dermatofibrosarcoma Trunk 15–19
Fat Liposarcoma Arms and Legs 15–19
Blood vessels Infantile hemangio-
pericytoma
Arms, legs, trunk, head, and neck Infant–4
Synovial tissue
(linings of joint cavities, tendon sheaths) 		Synovial sarcoma Legs, arms, and trunk 15–19
Peripheral nerves Malignant peripheral nerve sheath tumor (also called neurofibrosarcomas, malignant schwannomas, and neurogenic sarcomas) Arms, legs, and trunk 15–19
Muscular nerves Alveolar soft part sarcoma Arms and legs Infant–19
Cartilage and bone-forming tissue Extraskeletal myxoid chondrosarcoma Legs 10–14
Extraskeletal mesenchymal Legs 10–14

Types of sarcoma

(ICD-O codes are provided where available.)

Differentiating Sarcoma from Other Diseases

Soft tissue sarcoma needs to differentiated from soft tissue benign tumors such as:

Epidemiology and Demographics

Incidence

  • The estimated number of new cases of soft tissue sarcoma in the United States is approximately 12,000.[4]

Age

Percent Distribution of Soft Tissue Sarcoma by Histology

Risk Factors

Screening

  • There is insufficient evidence to recommend routine screening for sarcoma.

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

  • There are no ECG findings associated with sarcoma.

X-ray

Echocardiography or Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

  • There are no other diagnostic studies associated with sarcoma.

Treatment

Medical Therapy

Surgery

  • Surgery is the most common treatment for soft tissue sarcomas.
  • Depending on the size and location of the sarcoma, it may occasionally be necessary to remove all or part of an arm or leg (amputation).
  • In most cases, limb-sparing surgery is an option to avoid amputating the arm or leg.
  • It is important to obtain a margin free of tumor to decrease the likelihood of local recurrence and give the best chance for eradication of the tumor.

Radiation Therapy

  • In some cases, it can be used to treat tumors that cannot be surgically removed.
  • In multiple studies, radiation therapy has been found to improve the rate of local control, but has not had any influence on overall survival.

Primary Prevention

Secondary Prevention

References

  1. ↑ Borden EC, Baker LH, Bell RS, Bramwell V, Demetri GD, Eisenberg BL, Fletcher CD, Fletcher JA, Ladanyi M, Meltzer P, O'Sullivan B, Parkinson DR, Pisters PW, Saxman S, Singer S, Sundaram M, van Oosterom AT, Verweij J, Waalen J, Weiss SW, Brennan MF. Soft tissue sarcomas of adults: state of the translational science.Clin Cancer Res. 2003 Jun;9(6):1941-56. Review. PMID 12796356
  2. ↑ Tran T, Davila JA, El-Serag HB. The epidemiology of malignant gastrointestinal stromal tumors: an analysis of 1,458 cases from 1992 to 2000. Am J Gastroenterol 2005;100:162-8. PMID 15654796
  3. ↑ Longhi A, Errani C, De Paolis M, Mercuri M, Bacci G. Primary bone osteosarcoma in the pediatric age: state of the art. Cancer Treat Rev. 2006;32:423-36. PMID 16860938
  4. ↑ American Cancer Society: Cancer Facts and Figures 2014. Atlanta, Ga: American Cancer Society, 2014. Available online . Last accessed May 21, 2014.
  5. ↑ Wibmer C, Leithner A, Zielonke N, Sperl M, Windhager R (2010). "Increasing incidence rates of soft tissue sarcomas? A population-based epidemiologic study and literature review". Ann Oncol. 21 (5): 1106–11. doi:10.1093/annonc/mdp415. PMID 19858086.
  6. ↑ 6.0 6.1 6.2 6.3 6.4 6.5 "Sarcoma".

External Links

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