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  1 Sarcoma 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. his is in contrast to carcinomas, !hich are of epithelial srcin ( breast, colon,  pancreas, and others). Classification arcomas are given a number of different names, based on the t#pe of tissue from !hichthe# arise. $or example, osteosarcoma arises from  bone, chondrosarcoma arises from cartilage, and leiom#osarcoma arises from smooth muscle. arcomas strike people in all age ranges, but the# are ver# rare, accounting for onl# 1% of all cases of cancer . &1  oft tissuesarcomas, such as leiom#osarcoma, chondrosarcoma, and gastrointestinal stromal tumor  (G), are more common in adults than in children. G is the most common form of sarcoma, !ith approximatel# ***+** cases per #ear in the -nited tates. &  his should becompared !ith breast cancer, !ith approximatel# **,*** cases per #ear in /orth America. & 0one sarcomas, such as osteosarcoma and !ing's sarcoma, are more common in children than in adults. hese tumors most commonl# strike adolescents and #oung adults bet!eenthe ages of 1 and . n addition to being named based on the tissue of srcin, sarcomas arealso assigned a grade, such as lo! grade or high grade. 2o! grade sarcomas are usuall#treated surgicall#, although sometimes radiation therap# or chemotherap# are used. 3igh grade sarcomas are more fre4uentl# treated !ith chemotherap#. ince these tumors are morelikel# to undergo metastasis (spreading to distant sites), these tumors are treated moreaggressivel#. 5hildhood sarcomas are almost al!a#s treated !ith a combination of surger#and chemotherap#, and radiation is fre4uentl# used as !ell. he recognition that childhoodsarcomas are sensitive to chemotherap# has dramaticall# improved the survival of patients.$or example, in the era before chemotherap#, long term survival for patients !ith locali6edosteosarcoma !as onl# approximatel# *%, but no! has risen to 7*+8*%. &9 Types of sarcoma (5:+; codes are provided !here available.) ã oft tissue sarcoma, including o Alveolar soft part sarcoma (<=1>) o Angiosarcoma (<1*>) o 5#stosarcoma ?h#lloides &1  o :ermatofibrosarcoma (==>+==>) o :esmoid umor  (==1>1+==>1) o :esmoplastic small round cell tumor  (==*7>) o pithelioid arcoma (==*9>) o xtraskeletal chondrosarcoma (<*>) o xtraskeletal osteosarcoma (<1=*>) o $ibrosarcoma (==1*>) o 3emangioperic#toma (<1*) o 3emangiosarcoma (<1*>) o @aposi's sarcoma (<19*>) o 2eiom#osarcoma (==<*>+==<7>) o 2iposarcoma (==*>+===>) o 2#mphangiosarcoma (<18*+<18) o 2#mphosarcoma    o alignant fibrous histioc#toma (==*>) o  /eurofibrosarcoma (<9*>) o Bhabdom#osarcoma (=<**+=<*) o #novial sarcoma (<*9*>+<*9>) ã Askin's umor  (==*>) ã !ing's (<7*>) + ?/ (<98>) ã alignant 3emangioendothelioma (<1*>) ã alignant ch!annoma (<7*>+<71>) ã ;steosarcoma (<1=*>+<1<*>) ã 5hondrosarcoma (<*>+<9*>) Soft tissue sarcoma A soft tissue sarcoma  is a malignant (cancerous) tumor  that develops in mesench#mal tissue. esench#mal tissues encompass all the muscle, connective tissues, and  bones of the  bod#.oft tissue sarcomas can invade surrounding tissue and can metastasi6e (spread) to other organs of the bod#, forming secondar# tumors. he cells of secondar# tumors are similar tothose of the primar# (srcinal) cancer. econdar# tumors are referred to as metastatic softtissue sarcoma because the# are part of the same cancer and are not a ne! disease. he mostcommon site of spread is to the lungs.ome tumors of the soft tissue are benign (noncancerous) and are not sarcomas. hesetumors do not spread and are rarel# life+threatening. 3o!ever, benign tumors can cro!dnearb# organs and cause s#mptoms or interfere !ith normal bod# functions. Risk factors cientists do not full# understand !h# some people develop sarcomas !hile the vastmaCorit# does not. he vast maCorit# develop in patients !ith no kno!n risk factors or identifiable etiolog#. 3o!ever, b# identif#ing common characteristics in groups !ithunusuall# high occurrence rates, researchers have been able to single out some factors thatma# pla# a role in causing soft tissue sarcomas. ã tudies suggest that !orkers !ho are exposed to ?henox# herbicide inherbicides and chlorophenols in !ood preservatives ma# have an increased risk of developing soft tissue sarcomas. An unusual percentage of patients !ith a rare bloodvessel tumor, angiosarcoma of the liver, have been exposed to vin#l chloride in their !ork. his substance is used in the manufacture of certain plastics, notabl# ?D5. ã n the earl# 1<**s, !hen scientists !ere Cust discovering the potential uses of radiation to treat disease, little !as kno!n about safe dosage levels and precisemethods of deliver#. At that time, radiation !as used to treat a variet# of noncancerousmedical problems, including enlargement of the tonsils, adenoids, and th#mus gland.2ater, researchers found that high doses of radiation caused soft tissue sarcomas insome patients. 0ecause of this risk, radiation treatment for cancer is no! planned toensure that the maximum dosage of radiation is delivered to diseased tissue !hilesurrounding health# tissue is protected as much as possible. ã Besearchers believe that a retrovirus pla#s an indirect role in the development of @aposi's sarcoma, a rare cancer of the cells that line blood vessels in the skin andmucus membranes. @aposi's sarcoma often occurs in patients !ith A: (ac4uired   immune deficienc# s#ndrome). A:+related @aposi's sarcoma, ho!ever, has differentcharacteristics and is treated differentl# than t#pical soft tissue sarcomas. ã tudies have focused on genetic alterations that ma# lead to the development of soft tissue sarcomas. cientists have also found a small number of families in !hichmore than one member in the same generation has developed sarcoma. here havealso been reports of a fe! families in !hich relatives of children !ith sarcoma havedeveloped other forms of cancer at an unusuall# high rate. arcomas in these famil#clusters, !hich represent a ver# small fraction of all cases, ma# be related to a rareinherited genetic alteration of the p gene and is kno!n as 2i+$raumeni s#ndrome. ã 5ertain other inherited diseases are associated !ith an increased risk of developing soft tissue sarcomas. $or example, people !ith neurofibromatosis t#pe (also called von Becklinghausen's disease associated !ith alterations in the /$1 gene)are at an increased risk of developing soft tissue sarcomas kno!n as malignant peripheral nerve sheath tumors. ?atients !ith inherited retinoblastoma have alterationsin the B01 gene, a tumor suppressor gene, and are likel# to develop soft tissuesarcomas as the# mature into adulthood. Frequency oft tissue sarcomas are relativel# uncommon cancers. he# account for less than 1 % of all ne! cancer cases each #ear. he main reason is that soft tissue cells aren't constantl# fastdividing cells, but rather the opposite. $or instance, gro!th of muscles is made b#h#pertroph# of muscles cells rather than h#perplasia. n **7, about <,** ne! cases !ere diagnosed in the -nited tates. &1  oft tissuesarcomas are more commonl# found in older patients (E* #ears old) although in childrenand adolescents under age *, certain histologies are common (rhabdom#osarcoma). Symptoms n their earl# stages, soft tissue sarcomas usuall# do not cause s#mptoms. 0ecause softtissue is relativel# elastic, tumors can gro! rather large, pushing aside normal tissue, beforethe# are felt or cause an# problems. he first noticeable s#mptom is usuall# a painless lumpor s!elling. As the tumor gro!s, it ma# cause other s#mptoms, such as pain or soreness, as it presses against nearb# nerves and muscles. f in the abdomen it can cause abdominal painscommonl# mistaken for menstrual cramps, indigestion, or cause constipation. Diagnosis he onl# reliable !a# to determine !hether a soft tissue tumor is benign or malignant isthrough a biops#. herefore, all soft tissue lumps that persist or gro! should be biopsied. A biops# can be obtained via needle biops# or !ith surgical biops#. :uring this procedure, adoctor makes an incision or uses a special needle to remove a sample of tumor tissue. A pathologist examines the tissue under a microscope. f cancer is present, the pathologist canusuall# determine the t#pe of cancer and its grade. he grade of the tumor is determined b#ho! abnormal the cancer cells appear !hen examined under a microscope. he grade predicts the probable gro!th rate of the tumor and its tendenc# to spread. 2o!+gradesarcomas, although cancerous, are unlikel# to metastasi6e. 3igh+grade sarcomas are morelikel# to spread to other parts of the bod#. Treatment n general, treatment for soft tissue sarcomas depends on the stage of the cancer. hestage of the sarcoma is based on the si6e and grade of the tumor, and !hether the cancer has  9 spread to the l#mph nodes or other parts of the bod# (metastasi6ed). reatment options for soft tissue sarcomas include surger#, radiation therap#, and chemotherap#. ã urger# is the most common treatment for soft tissue sarcomas. f possible, thedoctor !ill remove the cancer and a safe margin of the health# tissue around it.:epending on the si6e and location of the sarcoma, it ma# occasionall# be necessar#to remove all or part of an arm or leg (amputation). 3o!ever, the need for amputationrarel# arisesF no more than 1* % to 1 % of individuals !ith sarcoma undergoamputation. n most cases, limb+sparing surger# is an option to avoid amputating thearm or leg. t is important to obtain a margin free of tumor to decrease the likelihoodof local recurrence and give the best chance for eradication of the tumor. ã Badiation therap# (treatment !ith x+ra#s or radioactive implants) ma# be usedeither before surger# to shrink tumors or after surger# to kill an# cancer cells that ma#have been left behind. n some cases, it can be used to treat tumours that cannot besurgicall# removed. n multiple studies, radiation therap# has been found to improvethe rate of local control, but has not had an# influence on overall survival. ã 5hemotherap# (treatment !ith anticancer drugs) ma# be used !ith radiationtherap# either before or after surger# to tr# to shrink the tumor or kill an# remainingcancer cells. n general, chemotherap# effects for soft tissue sarcoma have had littleimpact as opposed to other cancers. f the cancer has spread to other areas of the bod#,chemotherap# ma# be used to shrink tumors and reduce the pain and discomfort the#cause, but is unlikel# to eradicate the disease. he use of chemotherap# to prevent thespread of soft tissue sarcomas has not been proven to be effective. ?atients !ith softtissue sarcomas usuall# receive chemotherap# intravenousl# (inCected into a bloodvessel). :octors are conducting clinical trials in the hope of finding ne!, more effectivetreatments for soft tissue sarcomas, and better !a#s to use current treatments. 5linical trialsare in progress at hospitals and cancer centers around the countr#. 5linical trials are animportant part of the development of ne! methods of treatment. 0efore a ne! treatment can be recommended for general use, doctors conduct clinical trials to find out !hether thetreatment is safe for patients and effective against the disease. Table 1: Major Types of Soft Tissue Sarcomas in AultsTissue of !riginType of Cancer sual #ocation int$e %oyFibrous tissue $ibrosarcomaArms, legs, trunk alignant fibroush#stioc#toma2egs:ermatofibrosarcomarunk  Fat 2iposarcomaArms, legs, trunk  Muscle triated muscle mooth muscle Bhabdom#osarcoma 2eiom#osarcomaArms, legs -terus, digestivetract %loo &essels 3emangiosarcomaArms, legs, trunk 
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