Types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq

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Ukwelashwa kwe-Childhood Soft Tissue Sarcoma Treatment (®) -Patient Version

Imininingwane ejwayelekile Mayelana Nesicubu Esithambile Sethambo Sarcoma

AMAPHUZU ASEMQOKA

  • I-sarcoma yezicubu ezithambile zobuntwana yisifo lapho amangqamuzana amabi (umdlavuza) akha izicubu ezithambile zomzimba.
  • I-sarcoma ethambile yezicubu ivela ezinganeni nakubantu abadala.
  • Ukuba nezifo ezithile nokuphazamiseka okuzuzwe njengefa kungakhuphula ubungozi be-sarcoma yezicubu ezithambile zobuntwana.
  • Uphawu oluvame kakhulu lobuntwana bezicubu ezithambile sarcoma yisigaxa esingenabuhlungu noma ukuvuvukala kwezicubu ezithambile zomzimba.
  • Ukuhlolwa kokuxilonga kusetshenziselwa ukuthola (ukuthola) nokuxilonga i-sarcoma yezicubu ezithambile zobuntwana.
  • Uma ukuhlolwa kukhombisa ukuthi kungahle kube ne-sarcoma ethambile yezicubu, kwenziwa i-biopsy.
  • Kunezinhlobo eziningi ezahlukahlukene zama-sarcomas wezicubu ezithambile.
  • Izicubu zomzimba ezinonile
  • Izicubu zamathambo noqwanga
  • Izicubu zomzimba ezixakile
  • Izicubu zemisipha yamathambo
  • Izicubu zemisipha ebushelelezi
  • Okuthiwa izicubu ze-fibrohistiocytic
  • Izimila zemithambo yegazi
  • Izimila ze-Pericytic (Perivascular)
  • Amathumba wesizinda esingaziwa
  • Izimila zomthambo wegazi
  • Izici ezithile zithinta ukubikezela (ithuba lokululama) kanye nezinketho zokwelashwa.

I-sarcoma yezicubu ezithambile zobuntwana yisifo lapho amangqamuzana amabi (umdlavuza) akha izicubu ezithambile zomzimba.

Izicubu ezithambile zomzimba ziyaxhuma, zisekele, futhi zizungeze ezinye izitho zomzimba nezitho zomzimba. Izicubu ezithambile zifaka okulandelayo:

  • Amafutha.
  • Ukuhlanganiswa kwethambo noqwanga.
  • Izicubu eziyindilinga.
  • Imisipha.
  • Imizwa.
  • Ama-tendon (izibopho zezicubu ezixhuma imisipha emathanjeni).
  • Izicubu ze-Synovial (izicubu ezizungeze amalunga).
  • Imithambo yegazi.
  • Imikhumbi yeLymph.

I-sarcoma ethambile yezicubu ingatholakala noma kuphi emzimbeni. Ezinganeni, izicubu zakheka kakhulu ezingalweni, emilenzeni, esifubeni noma esiswini.

Izicubu ezithambile ze-sarcoma ezicutshini ezithambile zomzimba, kufaka phakathi imisipha, imisipha, amanoni, imithambo yegazi, imithambo ye-lymph, izinzwa nezicubu ezizungeze amalunga.

I-sarcoma ethambile yezicubu ivela ezinganeni nakubantu abadala.

I-sarcoma ethambile yezicubu ezinganeni ingaphendula ngokuhlukile ekwelashweni, futhi ingahle ibe nesibikezelo esingcono kune-sarcoma ethambile yezicubu kubantu abadala. (Bheka isifinyezo se- ku-Adult Soft Tissue Sarcoma Treatment ukuthola imininingwane yokwelashwa kubantu abadala.)

Ukuba nezifo ezithile nokuphazamiseka okuzuzwe njengefa kungakhuphula ubungozi be-sarcoma yezicubu ezithambile zobuntwana.

Noma yini enyusa ubungozi bokuthola isifo ibizwa ngokuthi ingozi. Ukuba nengcuphe akusho ukuthi uzothola umdlavuza; ukungabi nezici zobungozi akusho ukuthi ngeke uthole umdlavuza. Khuluma nodokotela wengane yakho uma ucabanga ukuthi ingane yakho ingaba sengcupheni.

Izici zobungozi besikhumba sarcoma sezicubu ezithambile zifaka ukuba nezinkinga ezilandelayo ezizuzwe njengefa:

  • Isifo se-Li-Fraumeni.
  • I-adenomatous polyposis (FAP) ejwayelekile.
  • Izinguquko zofuzo ze-RB1.
  • Izinguquko zofuzo ze-SMARCB1 (INI1).
  • Uhlobo lwe-Neurofibromatosis 1 (NF1).
  • Isifo seWerner.
  • Isifo sokuqina kwemithambo (tuberous sclerosis).
  • I-Adenosine deaminase-deficiated immunodeficiency eqinile ehlangene.

Ezinye izinto ezinobungozi zibandakanya okulandelayo:

  • Ukwelashwa kwangaphambilini ngemithi yokwelashwa ngemisebe.
  • Ukuba nengculazi (i-immune defence syndrome) kanye nokutheleleka ngegciwane le-Epstein-Barr ngasikhathi sinye.

Uphawu oluvame kakhulu lobuntwana bezicubu ezithambile sarcoma yisigaxa esingenabuhlungu noma ukuvuvukala kwezicubu ezithambile zomzimba.

I-sarcoma ingavela njengesigaxa esingenabuhlungu ngaphansi kwesikhumba, imvamisa engalweni, emlenzeni, esifubeni noma esiswini. Kungase kungabi khona ezinye izimpawu noma izimpawu ekuqaleni. Njengoba i-sarcoma ikhula futhi icindezela ezithweni eziseduze, izinzwa, imisipha, noma imithambo yegazi, ingadala izimpawu noma izimpawu, ezinjengobuhlungu noma ubuthakathaka.

Ezinye izimo zingadala izimpawu nezimpawu ezifanayo. Buza kudokotela wengane yakho ukuthi ingane yakho inayo yalezi zinkinga.

Ukuhlolwa kokuxilonga kusetshenziselwa ukuthola (ukuthola) nokuxilonga i-sarcoma yezicubu ezithambile zobuntwana.

Lezi zivivinyo nezinqubo ezilandelayo zingasetshenziswa:

  • Ukuhlolwa komzimba kanye nomlando: Ukuhlolwa komzimba ukuhlola izimpawu ezijwayelekile zempilo, kufaka phakathi nokuhlola izimpawu zezifo, njengezigaxa noma yini enye ebonakala ingajwayelekile. Kuzothathwa nomlando wemikhuba yezempilo yesiguli kanye nokugula nokwelashwa kwangaphambilini.
  • I-X-ray: I-x-ray uhlobo lwesigongolo samandla esingangena emzimbeni kwifilimu, senze izithombe zezindawo ezingaphakathi komzimba.
  • I-MRI (imaging resonance imaging): Inqubo esebenzisa uzibuthe, amagagasi omsakazo, nekhompyutha ukwenza uchungechunge lwezithombe ezinemininingwane yezindawo zomzimba, ezifana nesifuba, isisu, izingalo noma imilenze. Le nqubo ibizwa nangokuthi i-nuclear magnetic resonance imaging (i-NMRI).
I-Magnetic resonance imaging (MRI) yesisu. Ingane ilele etafuleni elingena kuskena seMRI, esithwebula izithombe zangaphakathi komzimba. Iphedi esiswini sengane isiza ukwenza izithombe zicace bha.
  • I-CT scan (i-CAT scan): Inqubo eyenza uchungechunge lwezithombe eziningiliziwe zezindawo ezingaphakathi komzimba, njengesifuba noma isisu, ezithathwe ezinhlangothini ezahlukene. Izithombe zenziwa yikhompyutha exhunywe kumshini we-x-ray. Idayi ingajovwa emthanjeni noma igwinywe ukusiza izitho noma izicubu zivele kahle. Le nqubo ibizwa nangokuthi i-computed tomography, i-computerized tomography, noma i-computer axial tomography.
Iskena se-computed tomography (CT) sesisu. Ingane ilele etafuleni elishibilika ngesithwebuli se-CT, esithatha izithombe ze-x-ray zangaphakathi kwesisu.
  • Ukuhlolwa kwe-Ultrasound: Inqubo lapho amaza omsindo wamandla aphezulu (i-ultrasound) ehlaselwa izicubu zangaphakathi noma izitho bese enza ama-echoes. Ama-echoes akha isithombe sezicubu zomzimba esibizwa nge-sonogram. Isithombe singaphrintwa ukuze sibhekwe ngokuhamba kwesikhathi.

Uma ukuhlolwa kukhombisa ukuthi kungahle kube ne-sarcoma ethambile yezicubu, kwenziwa i-biopsy.

Uhlobo lwe-biopsy luxhomeke, ngokwengxenye, ngosayizi wesisindo nokuthi ngabe liseduze yini nobuso besikhumba noma lujule ezicutshini. Olunye lwezinhlobo ezilandelayo ze-biopsies luvame ukusetshenziswa:

  • I-Core needle biopsy: Ukususwa kwezicubu kusetshenziswa inaliti ebanzi. Kuthathwa amasampula wezicubu eziningi. Le nqubo ingaqondiswa kusetshenziswa i-ultrasound, i-CT scan, noma i-MRI.
  • I-incisional biopsy: Ukususwa kwengxenye yesigaxa noma isampula lezicubu.
  • I-Biopsy e-Excisional: Ukususwa kwesigaxa sonke noma indawo yezicubu ezingabonakali zijwayelekile. Isazi sezifo sibheka izicubu ngaphansi kwesibonakhulu ukubheka amangqamuzana omdlavuza. I-biopsy ethandekayo ingasetshenziswa ukususa ngokuphelele izicubu ezincane eziseduze nobuso besikhumba. Lolu hlobo lwe-biopsy aluvamile ukusetshenziswa ngoba amangqamuzana omdlavuza angahlala ngemuva kwe-biopsy. Uma amangqamuzana omdlavuza ahlala, umdlavuza ungabuya noma usabalalele kwezinye izingxenye zomzimba.

I-MRI yesimila yenziwa ngaphambi kwe-biopsy ye-excisional. Lokhu kwenzelwa ukukhombisa ukuthi isimila sokuqala sakheka kuphi futhi singasetshenziswa ukuhola ukuhlinzwa esikhathini esizayo noma ukwelashwa ngemisebe.

Uma kunokwenzeka, udokotela ohlinzayo ozosusa noma yisiphi isimila esitholakalayo kufanele abambe iqhaza ekuhleleni i-biopsy. Ukubekwa kwezinaliti noma ukusikwa kwe-biopsy kungathinta ukuthi ngabe sonke isimila singasuswa ngesikhathi sokuhlinzwa kamuva.

Ukuhlela ukwelashwa okuhle kakhulu, isampula lezicubu ezisuswe ngesikhathi se-biopsy kumele libe likhulu ngokwanele ukuthola uhlobo lwe-sarcoma ethambile yezicubu futhi lenze ezinye izivivinyo zaselebhu. Amasampula amathishu azothathwa kwisimila sokuqala, ama-lymph node, nakwezinye izindawo ezingaba namaseli womdlavuza. Isazi sezifo sibheka izicubu ngaphansi kwesibonakhulu ukuze sibheke amangqamuzana omdlavuza futhi sithole uhlobo nobungako besimila. Izinga lesimila lincike ekutheni amangqamuzana omdlavuza abukeka ngendlela engavamile kanjani ngaphansi kwesibonakhulu nokuthi amaseli ahlukana ngokushesha kangakanani. Izicubu ezisezingeni eliphakeme naphakathi naphakathi zivame ukukhula futhi zisabalale ngokushesha okukhulu kunezimila ezisezingeni eliphansi.

Ngenxa yokuthi izicubu ezithambile ze-sarcoma kungaba nzima ukuyixilonga, isampula lezicubu kufanele lihlolwe ngudokotela wezifo onolwazi lokuthola isifo sarcoma esithambile.

Ukuhlolwa okukodwa noma okungaphezulu kokulandelayo kwelabhorethri kungenziwa ukutadisha amasampula wezicubu

  • Ukuhlolwa kwamangqamuzana: Ukuhlolwa kwelabhorethri ukubheka izakhi zofuzo ezithile, amaprotheni, noma amanye ama-molecule kusampula lezicubu, igazi, noma olunye uketshezi lomzimba. Ukuhlolwa kwamangqamuzana kungenziwa ngezinye izinqubo, ezinjenge-biopsies, ukusiza ukuthola ezinye izinhlobo zomdlavuza. Ukuhlolwa kwamangqamuzana kubheka ushintsho oluthile lofuzo noma lwe-chromosome olwenzeka kuma-sarcomas athile athambile.
  • Reverse transcription – polymerase chain reaction (RT – PCR) test: Isivivinyo selabhorethri lapho kulinganiswa inani lezinto zofuzo ezibizwa nge-mRNA ezenziwe ngofuzo oluthile. Kusetshenziswa i-enzyme ebizwa nge-reverse transcriptase ukuguqula ucezu oluthile lwe-RNA lube ucezu olufanayo lwe-DNA, olungakhuliswa (lwenziwe ngamanani amakhulu) ngenye i-enzyme ebizwa nge-DNA polymerase. Amakhophi we-DNA akhulisiwe asiza ukusho ukuthi ngabe i-mRNA ethile yenziwa ngofuzo. I-RT – PCR ingasetshenziswa ukuhlola ukwenziwa kwezakhi zofuzo ezithile ezingakhombisa ukuba khona kwamangqamuzana omdlavuza. Lokhu kuhlolwa kungasetshenziselwa ukubheka ushintsho oluthile ku-gene noma i-chromosome, engasiza ukuthola umdlavuza.
  • Ukuhlaziywa kwe-cytogenetic: Ukuhlolwa kwelabhoratri lapho ama-chromosomes wamaseli esampula yesicubu se-tumor abalwa futhi ahlolwe noma yiziphi izinguquko, ezinjengama-chromosomes aphukile, alahlekile, ahlelwe kabusha, noma amanye. Izinguquko kuma-chromosomes athile kungaba luphawu lomdlavuza. Ukuhlaziywa kwe-cytogenetic kusetshenziselwa ukuxilonga umdlavuza, ukuhlela ukwelashwa, noma ukuthola ukuthi ukwelashwa kusebenza kahle kangakanani. I-Fluorescence in situ hybridization (FISH) uhlobo lokuhlaziywa kwe-cytogenetic.
  • I-Immunocytochemistry: Isivivinyo selabhorethri esisebenzisa amasosha omzimba ukubheka ama-antigen athile (omaka) kusampula lamaseli wesiguli. Amasosha omzimba avamise ukuhlanganiswa ne-enzyme noma udayi we-fluorescent. Ngemuva kokuthi ama-antibody abophele kwi-antigen kusampula yamaseli wesiguli, i-enzyme noma idayi iyasebenza, bese kuthi i-antigen ibonakale ngaphansi kwesibonakhulu. Lolu hlobo lokuhlola lungasetshenziselwa ukutshela umehluko phakathi kwezinhlobo ezahlukahlukene ze-sarcoma yezicubu ezithambile.
  • I-light and electron microscopy: Isivivinyo selabhorethri lapho amaseli esampula yezicubu abukwa khona ngaphansi kwama-microscopes avamile futhi anamandla aphezulu ukubheka ushintsho oluthile kumaseli.

Kunezinhlobo eziningi ezahlukahlukene zama-sarcomas wezicubu ezithambile.

Amaseli ohlobo ngalunye lwe-sarcoma abukeka ehlukile ngaphansi kwesibonakhulu. Izicubu ezithambile zezicubu zihlelwe ngokususelwa ohlotsheni lweseli lezicubu ezithambile lapho zaqala khona ukwakheka.

Lesi sifinyezo simayelana nezinhlobo ezilandelayo ze-sarcoma yezicubu ezithambile:

Izicubu zomzimba ezinonile

I-Liposarcoma. Lona ngumdlavuza wamaseli anonile. I-Liposarcoma ivame ukwakheka kungqimba lwamafutha ngaphansi kwesikhumba. Ezinganeni nasebusheni, i-liposarcoma imvamisa ibanga eliphansi (kungenzeka likhule futhi lisakazeke kancane). Kunezinhlobo ezahlukahlukene ze-liposarcoma, kufaka phakathi:

  • I-myxoid liposarcoma. Lokhu kuvame ukuba ngumdlavuza osezingeni eliphansi ophendula kahle ekwelashweni.
  • I-lipomarcic liposarcoma. Lokhu kuvame ukuba ngumdlavuza osezingeni eliphakeme okungenzeki ukuthi uphendule kahle ekwelashweni.

Izicubu zamathambo noqwanga

Izicubu zamathambo noqwanga ziyingxube yamaseli amathambo namaseli eqwanga. Izicubu zamathambo noqwanga zihlanganisa izinhlobo ezilandelayo:

  • I-extraceletelet mesenchymal chondrosarcoma. Lolu hlobo lwesimila samathambo noqwanga luvame ukuthinta abantu abadala abasebasha futhi lwenzeka ekhanda nasentanyeni.
  • I-osteosarcoma eyengeziwe. Lolu hlobo lwesimila samathambo noqwanga alutholakali ezinganeni nasebusheni. Kungenzeka ibuye ngemuva kokwelashwa futhi ingasakazeka emaphashini.

Izicubu zomzimba ezixakile

Izicubu zomzimba ezihlanganisiwe zifaka izinhlobo ezilandelayo:

  • Uhlobo lwe-Desmoid-fibromatosis (olubizwa nangokuthi i-desmoid tumor noma i-fibromatosis enobudlova). Lesi simila sezicubu esinemicu sisezingeni eliphansi (kungenzeka likhule kancane). Ingabuya izicubu eziseduze kepha imvamisa ayisabalali iye ezingxenyeni ezikude zomzimba. Kwesinye isikhathi uhlobo lwe-desmoid-fibromatosis lungayeka ukukhula isikhathi eside. Kuyaqabukela, isimila singanyamalala ngaphandle kokwelashwa.

Izicubu ze-Desmoid kwesinye isikhathi zenzeka ezinganeni ezinezinguquko kuhlobo lwe-APC. Izinguquko kulesakhi zofuzo zingadala nomndeni adenomatous polyposis (FAP). I-FAP yisimo esizuzwe njengefa (esidluliselwe kusuka kubazali kuya enzalweni) lapho ama-polyps amaningi (ukukhula ezinhlakeni zomlomo) akha ezindongeni zangaphakathi zekoloni kanye nengxenyeni. Ukwelulekwa ngofuzo (ingxoxo nochwepheshe oqeqeshiwe mayelana nezifo ezizuzwe njengefa kanye nezinketho zokuhlolwa kofuzo) kungadingeka.

  • Ama-protuberans eDermatofibrosarcoma. Lesi isimila sezingqimba ezijulile zesikhumba esivame ukwakheka esiqwini, ezingalweni noma emilenzeni. Amaseli alesi sifo anoshintsho oluthile lofuzo olubizwa ngokuthi ukuhanjiswa (ingxenye yesakhi sofuzo seCOL1A1 indawo enengxenye yofuzo lwe-PDGFRB). Ukuxilonga i-dermatofibrosarcoma protuberans, amaseli wesimila ahlolwe lolu shintsho lofuzo. I-Dermatofibrosarcoma protuberans imvamisa ayisabalali kuma-lymph node noma ezinye izingxenye zomzimba.
  • Isimila esivuthayo se-myofibroblastic. Lo mdlavuza wakhiwe ngamangqamuzana emisipha, amaseli wezicubu axhuma, namaseli athile omzimba. Kwenzeka ezinganeni nasebusheni. Imvamisa yakha izicubu ezithambile, amaphaphu, ubende nesifuba. Ibuya njalo ngemuva kokwelashwa kepha kuyaqabukela isabalale iye ezingxenyeni ezikude zomzimba. Kutholakale ushintsho oluthile kwezofuzo cishe engxenyeni yalezi zicubu.

I-Fibrosarcoma.

Kunezinhlobo ezimbili ze-fibrosarcoma ezinganeni nasebusheni:

  • I-Infantile fibrosarcoma (ebizwa nangokuthi i-congenital fibrosarcoma). Lolu hlobo lwe-fibrosarcoma luvame ukwenzeka ezinganeni ezineminyaka engu-1 nangaphansi futhi lungabonakala ekuhlolweni kokubeletha kwe-ultrasound. Lesi isimila sikhula ngokushesha futhi kaningi sikhulu ekuxilongweni. Akuvamile ukusakazeka ezingxenyeni ezikude zomzimba. Amaseli alesi sifo ngokuvamile anoshintsho oluthile lwezakhi zofuzo olubizwa ngokuthi ukuhanjiswa (ingxenye yoshintsho olulodwa lwe-chromosome ibeka ingxenye yengxenye ye-chromosome). Ukuthola i-infantile fibrosarcoma, amaseli wesimila ahlolwe lolu shintsho lofuzo. Isigaxa esifanayo siye sabonakala ezinganeni ezindala, kepha asinayo i-translocation evame ukubonakala ezinganeni ezincane.
  • I-fibrosarcoma yabantu abadala. Lolu uhlobo olufanayo lwe-fibrosarcoma etholakala kubantu abadala. Amaseli alesi sifo awanawo ushintsho lofuzo olutholakala ku-infantile fibrosarcoma. (Bheka isifinyezo se- ku-Adult Soft Tissue Sarcoma Treatment ukuthola eminye imininingwane.)
  • I-Myxofibrosarcoma. Lesi isimila sezicubu esingajwayelekile esivela kancane ezinganeni kunabantu abadala.
  • I-low-grade fibromyxoid sarcoma. Lesi isimila esikhula kancane esakha ngokujulile ezingalweni noma emilenzeni futhi sithinta kakhulu abantu abadala nabaseminyakeni yobudala ephakathi. Isimila singabuya emuva kweminyaka eminingi selashwe bese sisakazeka emaphashini nasendlini yodonga lwesifuba. Ukulandelwa impilo yonke kuyadingeka.
  • Ukushaya i-epithelioid fibrosarcoma. Lesi isimila sezicubu esingajwayelekile esikhula ngokushesha. Ingabuya futhi isabalale kwezinye izingxenye zomzimba eminyakeni ngemuva kokwelashwa. Ukulandelelwa kwesikhathi eside kuyadingeka.

Izicubu zemisipha yamathambo

Imisipha yamathambo inamathele emathanjeni futhi isiza ukuhamba komzimba.

  • I-Rhabdomyosarcoma. I-Rhabdomyosarcoma yisona sarcoma esivame kakhulu ebuntwaneni esithambile ezinganeni ezineminyaka engu-14 nangaphansi. (Bheka isifinyezo se- ku-Childhood Rhabdomyosarcoma Treatment ukuthola eminye imininingwane.)

Izicubu zemisipha ebushelelezi

Imisipha ebushelelezi ingaphakathi kwemithambo yegazi nezitho zangaphakathi eziyize ezifana nesisu, amathumbu, isinye nesibeletho.

  • I-Leiomyosarcoma. Lesi simila esibushelelezi semisipha sixhunyaniswe ne-Epstein-Barr virus ezinganeni ezine-HIV noma i-AIDS. I-Leiomyosarcoma nayo ingakha njengomdlavuza wesibili kwabasindile be-retinoblastoma ezuzwe njengefa, kwesinye isikhathi iminyaka eminingi ngemuva kokwelashwa kokuqala kwe-retinoblastoma.

Okuthiwa izicubu ze-fibrohistiocytic

  • Isimila se-Plexiform fibrohistiocytic. Lesi isimila esingavamile esivame ukuthinta izingane nabantu abadala abasebasha. Isimila ngokuvamile siqala njengokukhula okungenabuhlungu ngaphakathi noma ngaphansi kwesikhumba engalweni, esandleni, noma esihlakaleni. Kungavame ukusabalala kuma-lymph node aseduze noma emaphashini.

Izimila zemithambo yegazi

Umgogodla wezinzwa wenziwa izingqimba zokuvikela i-myelin ezihlanganisa amangqamuzana emizwa engeyona ingxenye yobuchopho noma umgogodla. Izicubu zemithambo yegazi zifaka izinhlobo ezilandelayo:

  • Isigaxa semithambo esibuhlungu esibuhlungu. Ezinye izingane ezine-peripheral nerve sheath tumor enesifo esingajwayelekile sofuzo esibizwa nge-neurofibromatosis type 1 (NF1). Lesi simila singaba sezingeni eliphansi noma ebangeni eliphakeme.
  • Isimila esibi seTriton . Lezi izicubu ezikhula ngokushesha kakhulu ezenzeka kaningi ezinganeni ezine-NF1.
  • I-Ectomesenchymoma. Lesi isimila esikhula ngokushesha esenzeka ikakhulukazi ezinganeni. Ama-Ectomesenchymomas angakha esokeni leso, isisu, izingalo noma imilenze.

Izimila ze-Pericytic (Perivascular)

Izicubu ze-Pericytic zakha amaseli asonga imithambo yegazi. Izicubu ze-Pericytic zifaka izinhlobo ezilandelayo:

  • I-Myopericytoma. I-Infantile hemangiopericytoma uhlobo lwe-myopericytoma. Izingane ezingaphansi konyaka ongu-1 ngesikhathi sokuxilongwa zingase zibikezele kangcono. Ezigulini ezingaphezulu konyaka owodwa, i-infantile hemangiopericytoma inamathuba amaningi okusabalala kwezinye izingxenye zomzimba, kufaka phakathi ama-lymph node namaphaphu.
  • I-myofibromatosis yezinsana. I-myofibromatosis yezinsana olunye uhlobo lwe-myopericytoma. Kuyisigaxa esinemicu esivame ukwenzeka eminyakeni emibili yokuqala yokuphila. Kungaba ne-nodule eyodwa ngaphansi kwesikhumba, imvamisa endaweni yekhanda nentamo (i-myofibroma), noma amaqhubu amaningana esikhunjeni, emisipheni, noma ethanjeni (i-myofibromatosis). Ezigulini ezine-myofibromatosis yezinsana, umdlavuza ungasakazeka nasezithweni. Lezi zicubu zingaphela ngaphandle kokwelashwa.

Amathumba wesizinda esingaziwa

Izimila zemvelaphi engaziwa yeseli (uhlobo lweseli isimila sokuqala esakhiwe kulo alwaziwa) zifaka izinhlobo ezilandelayo:

  • I-Synovial sarcoma. I-Synovial sarcoma uhlobo oluvamile lwe-sarcoma ethambile yezicubu ezinganeni nasebusheni. Imvamisa yakha izicubu ezizungeze amalunga ezingalweni noma emilenzeni, kepha futhi ingakheka esiqwini, ekhanda, noma entanyeni. Amaseli alesi sifo ngokuvamile anoshintsho oluthile lwezakhi zofuzo olubizwa ngokuthi ukuhanjiswa (ingxenye yoshintsho olulodwa lwe-chromosome ibeka ingxenye yengxenye ye-chromosome). Izicubu ezinkulu zinobungozi obukhulu bokusabalala kwezinye izingxenye zomzimba, kufaka phakathi amaphaphu. Izingane ezingaphansi kweminyaka eyi-10 isimila sazo singamasentimitha ama-5 noma sincane futhi esakhe ezingalweni noma emilenzeni sinesibikezelo esingcono.
  • I-Epithelioid sarcoma. Lesi yisarcoma esingajwayelekile esivame ukuqala ngokujula kwezicubu ezithambile njengesigaxa esikhula kancane, esiqinile futhi singasabalala kuma-lymph node. Uma umdlavuza owakhiwe ezingalweni, emilenzeni, noma ezinqeni, kungenziwa i-sentinel lymph node biopsy ukuhlola umdlavuza kuma-lymph node.
  • Ingxenye ethambile ye-alveolar sarcoma. Lesi isimila esingajwayelekile sezicubu ezithambile ezisekelayo ezixhuma futhi zizungeze izitho nezinye izicubu. Kwenzeka kakhulu ezingalweni nasemilenzeni kepha kungenzeka ezicutshini zomlomo, emihlathini nasebusweni. Ingakhula kancane futhi ivame ukusabalala kwezinye izingxenye zomzimba. Ingxenye ethambile ye-alveolar sarcoma ingahle ibe nokuchasisa okungcono lapho isimila singamasentimitha ama-5 noma sincane noma lapho isimila sisuswe ngokuphelele ngokuhlinzwa. Amaseli alesi sifo ngokuvamile anoshintsho oluthile lofuzo olubizwa ngokuthi ukuhanjiswa (ingxenye yesakhi sofuzo se-ASSPL indawo enengxenye yofuzo lwe-TFE3). Ukuhlonza i-alveolar soft part sarcoma, amaseli wesimila ahlolwe lolu shintsho lofuzo.
  • Sula i-cell sarcoma yezicubu ezithambile. Lesi isimila sesicubu esithambile esikhula kancane esiqala kuthenda (izicubu ezinzima, ezinezintambo, njengezintambo ezixhuma imisipha nethambo noma kwenye ingxenye yomzimba). I-cell sarcoma ecacile ivame ukwenzeka ezicutshini ezijulile zonyawo, isithende kanye neqakala. Ingasakazeka kuma-lymph node aseduze. Amaseli alesi simila ngokuvamile anoshintsho oluthile lwezakhi zofuzo olubizwa ngokuthi ukuhanjiswa (ingxenye yesakhi sofuzo se-EWSR1 indawo enengxenye yesakhi sofuzo i-ATF1 noma i-CREB1). Ukuhlonza i-cell sarcoma ecacile yezicubu ezithambile, amaseli wesimila ahlolwe lolu shintsho lofuzo.
  • I-extrondeletal myxoid chondrosarcoma. Lolu hlobo lwe-sarcoma yezicubu ezithambile kungenzeka ezinganeni nasebusheni. Ngokuhamba kwesikhathi, ijwayele ukusakazeka kwezinye izingxenye zomzimba, kufaka phakathi ama-lymph node namaphaphu. Isimila singabuya emuva kweminyaka eminingi selashwe.
  • I-Extraskeletal Ewing sarcoma. Bona isifinyezo se- ku-Ewing Sarcoma Treatment ukuthola imininingwane.
  • I-Desmoplastic small cell cell isimila. Lesi simila sivame ukwakheka ku-peritoneum esiswini, okhalweni, kanye / noma kwi-peritoneum esiya esikhwameni, kepha singakheka ezinso noma kwezinye izitho eziqinile. Izinqwaba zezimila ezincane kungenzeka ku-peritoneum. Isimila seseli esiyindilinga esincane esincibilikayo singasakazeka nasemaphashini nakwezinye izingxenye zomzimba. Amaseli alesi sifo ngokuvamile anoshintsho oluthile lwezakhi zofuzo olubizwa ngokuthi ukuhanjiswa (ingxenye yoshintsho olulodwa lwe-chromosome ibeka ingxenye yengxenye ye-chromosome). Ukuhlonza i-desmoplastic small cell tumor, amaseli we-tumor ahlolwe lolu shintsho lofuzo.
  • Isigaxa se-rhabdoid esingeziwe (i-extracranial). Lesi simila esikhula ngokushesha sakha izicubu ezithambile njengesibindi nesinye. Imvamisa kwenzeka ezinganeni ezincane, kufaka phakathi izingane ezisanda kuzalwa, kepha kungenzeka ezinganeni ezindala nakubantu abadala. Izicubu zeRhabdoid zingaxhunyaniswa nokuguqulwa kwesakhi sofuzo sokucindezela isimila esibizwa ngeSMARCB1. Lolu hlobo lofuzo lwenza iprotheni esiza ukulawula ukukhula kwamaseli. Izinguquko kuhlobo lwe-SMARCB1 zingazuzwa njengefa. Ukwelulekwa ngofuzo (ingxoxo nochwepheshe oqeqeshiwe mayelana nezifo ezizuzwe njengefa kanye nesidingo sokuhlolwa kofuzo) kungadingeka.
  • Izicubu zeseli zePerivascular epithelioid (PEComas). I-Benign PEComas kungenzeka ezinganeni ezinesimo esizuzwe njengefa esibizwa nge-tuberous sclerosis. Zenzeka esiswini, emathunjini, emaphashini nasezitho zomzimba zofuzo. Ama-PEComas akhula kancane futhi iningi lawo kungenzeka lingasabalali.
  • I-sarcoma engahlukaniswanga / engahlonziwe. Lezi zicubu zivame ukwenzeka emathanjeni noma emisipheni eboshelwe emathanjeni futhi esiza umzimba ukuba unyakaze.
  • I-pleomorphic sarcoma / i-histiocytoma enobungozi (ibanga eliphakeme). Lolu hlobo lwesimila sezicubu ezithambile lungakheka ezingxenyeni zomzimba lapho iziguli zithole khona ukwelashwa ngemisebe esikhathini esedlule, noma njengomdlavuza wesibili ezinganeni ezine-retinoblastoma. Isimila ngokuvamile sakheka ezingalweni noma emilenzeni futhi singadlulela kwezinye izingxenye zomzimba. (Bheka isifinyezo se- ku-Osteosarcoma kanye ne-Malignant Fibrous Histiocytoma ye-Bone Treatment ukuthola imininingwane nge-malignant fibrous histiocytoma of bone.)

Izimila zomthambo wegazi

Izicubu zemithambo yegazi zifaka izinhlobo ezilandelayo:

  • I-Epithelioid hemangioendotheliomaoma. I-Epithelioid hemangioendotheliomas ingenzeka ezinganeni kepha zivame kakhulu kubantu abadala abaphakathi kweminyaka engama-30 nengama-50 ubudala. Imvamisa zenzeka esibindini, emaphashini noma ethanjeni. Kungenzeka zikhule ngokushesha noma zikhule kancane. Cishe kokuthathu kwamacala, isimila sisakazekela kwezinye izingxenye zomzimba ngokushesha okukhulu. (Bheka isifinyezo se- ku-Childhood Vascular Tumors Treatment ukuthola eminye imininingwane.)
  • I-Angiosarcoma yezicubu ezithambile. I-Angiosarcoma yezicubu ezithambile iyisimila esikhula ngokushesha esakha emithanjeni yegazi noma emithanjeni ye-lymph kunoma iyiphi ingxenye yomzimba. Ama-angiosarcomas amaningi aphakathi noma ngaphansi kwesikhumba. Labo abasemathisheni athambile ajulile bangakha esibindini, ebunzini, noma emaphashini. Zivame kakhulu ezinganeni, kwesinye isikhathi ezinesimila esingaphezu kwesisodwa esikhunjeni noma esibindini. Ngokuvamile, i-hemangioma yezinsana ingaba i-angiosarcoma yezicubu ezithambile. (Bheka isifinyezo se- ku-Childhood Vascular Tumors Treatment ukuthola eminye imininingwane.)

Bona izifinyezo ezilandelayo ze- ngolwazi mayelana nezinhlobo ze-sarcoma yezicubu ezithambile ezingafakiwe kulesi sifinyezo:

  • Ukwelashwa Kwe-Childhood Rhabdomyosarcoma
  • Ukwelashwa kwe-Ewing Sarcoma
  • I-Osteosarcoma neMalignant Fibrous Histiocytoma Yokwelashwa Kwamathambo
  • Umdlavuza Ongajwayelekile Wokwelashwa Kwezingane (izicubu zomzimba emathunjini)

Izici ezithile zithinta ukubikezela (ithuba lokululama) kanye nezinketho zokwelashwa.

Isibikezelo (ithuba lokululama) nezinketho zokwelashwa zincike kokulandelayo:

  • Ingxenye yomzimba lapho kwakhiwa khona isimila okokuqala.
  • Usayizi nebanga lesimila.
  • Uhlobo lwe-sarcoma yezicubu ezithambile.
  • Sijule kangakanani isimila ngaphansi kwesikhumba.
  • Ukuthi isimila sesisabalalele kwezinye izindawo emzimbeni nokuthi sisabalale kuphi.
  • Inani lesimila esisele ngemuva kokuhlinzwa ukusikhipha.
  • Ukuthi kwakusetshenziswa ukwelashwa ngemisebe ukwelapha isimila.
  • Ukuthi umdlavuza usanda kutholakala noma usuphindile (buyela emuva).

Izigaba ze-Soft Tissue Sarcoma Yezingane

AMAPHUZU ASEMQOKA

  • Ngemuva kokutholwa kwengane izicubu ezithambile zesarcoma, kwenziwa ukuhlolwa ukuze kutholakale ukuthi amangqamuzana omdlavuza asabalalele yini kwezinye izingxenye zomzimba.
  • Zintathu izindlela umdlavuza osakazeka ngayo emzimbeni.
  • Umdlavuza ungasakazeka usuka lapho uqale khona kwezinye izingxenye zomzimba.

Ngemuva kokutholwa kwengane izicubu ezithambile zesarcoma, kwenziwa ukuhlolwa ukuze kutholakale ukuthi amangqamuzana omdlavuza asabalalele yini kwezinye izingxenye zomzimba.

Inqubo esetshenziswayo ukuthola ukuthi umdlavuza ususabalele ngaphakathi kwezicubu ezithambile noma kwezinye izingxenye zomzimba ibizwa ngokuthi yi-staging. Akunasistimu ejwayelekile yokubeka izitaki zesarcoma yengane ethambile.

Ukuhlela ukwelashwa, kubalulekile ukwazi uhlobo lwe-sarcoma ethambile yezicubu, ukuthi isimila singasuswa yini ngokuhlinzwa, nokuthi umdlavuza ususabalele kwezinye izingxenye zomzimba.

Izinqubo ezilandelayo zingasetshenziswa ukuthola ukuthi umdlavuza ususakazekile yini:

  • I-Sentinel lymph node biopsy: Ukususwa kwe-sentinel lymph node ngesikhathi sokuhlinzwa. I-sentinel lymph node iyisakhi sokuqala se-lymph eqenjini lama-lymph node ukuthola i-lymphatic drainage evela ku-tumor yokuqala. Kuyi-lymph node yokuqala umdlavuza ongahle usabalale uye kusukela kusimila sokuqala. Kufakwa into enemisebe kanye / noma udayi oluhlaza okwesibhakabhaka eduze kwesimila. Into noma udayi ugeleza ngemigudu ye-lymph uye kuma-lymph node. I-lymph node yokuqala yokwamukela into noma udayi iyasuswa. Isazi sezifo sibheka izicubu ngaphansi kwesibonakhulu ukubheka amangqamuzana omdlavuza. Uma amangqamuzana omdlavuza engatholakali, kungahle kungadingeki ukususa ama-lymph node amaningi. Kwesinye isikhathi, i-sentinel lymph node itholakala eqenjini elingaphezu kwelilodwa lama-node. Le nqubo isetshenziselwa i-epithelioid ne-clear cell sarcoma.
  • I-CT scan (i-CAT scan): Inqubo eyenza uchungechunge lwezithombe ezinemininingwane yezindawo ezingaphakathi komzimba, njengesifuba, ezithathwe ezinhlangothini ezahlukene. Izithombe zenziwa yikhompyutha exhunywe kumshini we-x-ray. Idayi ingajovwa emthanjeni noma igwinywe ukusiza izitho noma izicubu zivele kahle. Le nqubo ibizwa nangokuthi i-computed tomography, i-computer ene-tomography, noma i-computer axial tomography.
  • Ukuskena kwe-PET: Ukuskena kwe- PET kuyindlela yokuthola amangqamuzana amabi esigaxa emzimbeni. Inani elincane le-glucose enemisebe (ushukela) lifakwa emthanjeni. Isithwebuli se-PET sizungeza umzimba futhi senze isithombe lapho i-glucose isetshenziswa khona emzimbeni. Amaseli amathumba amabi abonakala ekhanya esithombeni ngoba ayasebenza futhi athatha i-glucose eningi kunamaseli avamile. Le nqubo ibizwa nangokuthi i-positron emission tomography (PET) scan.
  • Iskena se-PET-CT: Inqubo ehlanganisa izithombe kusuka kuskena se-PET nokuskena kwe-computed tomography (CT). Izikena ze-PET ne-CT zenziwa ngasikhathi sinye emshinini ofanayo. Izithombe ezivela kuzo zombili izikena zihlanganisiwe ukwenza isithombe esinemininingwane eminingi kunalokho ukuhlolwa obekungakwenza ngokwakho.

Zintathu izindlela umdlavuza osakazeka ngayo emzimbeni.

Umdlavuza ungasakazeka ngezicubu, uhlelo lwe-lymph, kanye negazi:

  • Izicubu. Umdlavuza usakazeka lapho uqale khona ngokukhulela ezindaweni eziseduze.
  • Uhlelo lweLymph. Umdlavuza usakazeka lapho uqale khona ngokungena ohlelweni lwe-lymph. Umdlavuza udabula imithambo ye-lymph uye kwezinye izingxenye zomzimba.
  • Igazi. Umdlavuza usakazeka lapho uqale khona ngokungena egazini. Umdlavuza uhamba ngemithambo yegazi uye kwezinye izingxenye zomzimba.

Umdlavuza ungasakazeka usuka lapho uqale khona kwezinye izingxenye zomzimba.

Lapho umdlavuza usakazekela kolunye uhlangothi lomzimba, ubizwa ngokuthi yi-metastasis. Amaseli womdlavuza aqhamuka lapho aqale khona (isimila sokuqala) futhi ahamba ngohlelo lwe-lymph noma igazi.

  • Uhlelo lweLymph. Umdlavuza ungena ohlelweni lwe-lymph, udabule emithanjeni ye-lymph, bese wenza isimila (isimila se-metastatic) kwenye ingxenye yomzimba.
  • Igazi. Umdlavuza ungena egazini, udabule emithanjeni yegazi, bese wenza isimila (isimila se-metastatic) kwenye ingxenye yomzimba.

Isigaxa se-metastatic luhlobo olufanayo lomdlavuza njengesimila sokuqala. Isibonelo, uma izicubu ezithambile sarcoma zisakazeka ziye emaphashini, amangqamuzana omdlavuza asephashini angamaseli wezicubu ezithambile ze-sarcoma. Lesi sifo siyi-metastatic soft tissue sarcoma, hhayi umdlavuza wamaphaphu.

Izicubu Zesikhumba Esithambile Esiphindaphindayo Nezithuthukayo

I-sarcoma yezicubu ezithambile ebuntwaneni ngumdlavuza ophindaphindiwe (wabuya) ngemuva kokuphathwa. Umdlavuza kungenzeka ubuye endaweni efanayo noma kwezinye izingxenye zomzimba.

Ukuthuthuka kwezicubu ezithambile zobuntwana ngumdlavuza ongaphendulanga ekwelashweni.

Uhlolojikelele lwenketho yokwelashwa

AMAPHUZU ASEMQOKA

  • Kunezinhlobo ezahlukahlukene zokwelashwa kweziguli ezine-sarcoma yezicubu ezithambile zobuntwana.
  • Izingane ezine-sarcoma yezicubu ezithambile ebuntwaneni kufanele zihlelwe ukwelashwa kwazo yiqembu labahlinzeki bezokunakekelwa kwempilo abangochwepheshe ekwelapheni umdlavuza ezinganeni.
  • Ukwelashwa kwe-sarcoma yezicubu ezithambile ebuntwaneni kungadala imiphumela emibi.
  • Izinhlobo eziyisikhombisa zokwelashwa okujwayelekile zisetshenzisiwe:
  • Ukuhlinzwa
  • Ukwelashwa ngemisebe
  • Ukwelashwa ngamakhemikhali
  • Ukuqaphela
  • Ukwelashwa okuhlosiwe
  • I-Immunotherapy
  • Olunye Ukwelashwa Kwezidakamizwa
  • Izinhlobo ezintsha zokwelashwa ziyahlolwa ezivivinyweni zokwelashwa.
  • Ukwelashwa kwe-Gene
  • Iziguli zingafuna ukucabanga ngokubamba iqhaza esivivinyweni somtholampilo.
  • Iziguli zingangena ezivivinyweni zokwelashwa ngaphambi, ngesikhathi, noma ngemuva kokuqala ukwelashwa kwazo komdlavuza.
  • Ukuhlolwa kokulandelela kungadingeka.

Kunezinhlobo ezahlukahlukene zokwelashwa kweziguli ezine-sarcoma yezicubu ezithambile zobuntwana.

Izinhlobo ezahlukene zokwelashwa ziyatholakala ezigulini ezine-sarcoma yezicubu ezithambile zobuntwana. Ezinye izindlela zokwelapha zijwayelekile (indlela esetshenziswa njengamanje), kanti ezinye ziyahlolwa ezivivinyweni zokwelashwa. Isivivinyo somtholampilo wokwelashwa isifundo socwaningo esihloselwe ukusiza ukuthuthukisa ukwelashwa kwamanje noma ukuthola ulwazi ngezindlela zokwelashwa ezintsha zeziguli ezinomdlavuza. Lapho izivivinyo zokwelashwa zibonisa ukuthi ukwelashwa okusha kungcono kunokwelashwa okujwayelekile, ukwelashwa okusha kungaba ukwelashwa okujwayelekile.

Ngoba umdlavuza ezinganeni uyivelakancane, ukubamba iqhaza esivivinyweni somtholampilo kufanele kubhekwe. Ezinye izivivinyo zokwelashwa zivulekele kuphela iziguli ezingakaqali ukwelashwa.

Izingane ezine-sarcoma yezicubu ezithambile ebuntwaneni kufanele zihlelwe ukwelashwa kwazo yiqembu labahlinzeki bezokunakekelwa kwempilo abangochwepheshe ekwelapheni umdlavuza ezinganeni.

Ukwelashwa kuzobhekwa ngudokotela wezingane oncologist, udokotela ogxile ekwelapheni izingane ezinomdlavuza. I-oncologist yezingane isebenza nabanye abahlinzeki bokunakekelwa kwempilo abangochwepheshe ekwelapheni izingane ezinezicubu ezithambile ze-sarcoma futhi ezikhethekile ezindaweni ezithile zemithi. Lokhu kungafaka udokotela ohlinzayo wezingane ngokuqeqeshwa okukhethekile ekususweni kwama-sarcomas wezicubu ezithambile. Ochwepheshe abalandelayo bangafakwa futhi:

  • Udokotela wezingane.
  • I-radiation oncologist.
  • I-hematologist yezingane.
  • Umhlengikazi ongumhlengikazi.
  • Uchwepheshe wokuvuselelwa.
  • Isazi sokusebenza kwengqondo.
  • Usonhlala kahle.
  • Ingcweti kwezempilo yengane.

Ukwelashwa kwe-sarcoma yezicubu ezithambile ebuntwaneni kungadala imiphumela emibi.

Ngemininingwane ngemiphumela emibi eqala ukwelashwa komdlavuza, bheka ikhasi lethu le-Side Effects.

Imiphumela emibi yokwelashwa komdlavuza eqala ngemuva kokwelashwa futhi iqhubeke izinyanga noma iminyaka ibizwa ngemiphumela ephuzile. Imiphumela yamuva yokwelashwa komdlavuza ingafaka:

  • Izinkinga zomzimba.
  • Izinguquko zemizwa, imizwa, ukucabanga, ukufunda, noma inkumbulo.
  • Umdlavuza wesibili (izinhlobo ezintsha zomdlavuza).

Eminye imiphumela yesikhashana ingalashwa noma ilawulwe. Kubalulekile ukukhuluma nodokotela bengane yakho ngemiphumela yokwelashwa komdlavuza engaba nayo enganeni yakho. (Bheka isifinyezo se- ngemiphumela yamuva yokwelashwa komdlavuza wezingane ukuthola eminye imininingwane.)

Izinhlobo eziyisikhombisa zokwelashwa okujwayelekile zisetshenzisiwe:

Ukuhlinzwa

Ukuhlinzwa ukususa ngokuphelele i-sarcoma ethambile yezicubu kwenziwa lapho kungenzeka. Uma isimila sikhulu kakhulu, ukwelashwa ngemisebe noma i-chemotherapy kunganikezwa kuqala, ukwenza isimila sibe sincane futhi sinciphise inani lezicubu ezidinga ukususwa ngesikhathi sokuhlinzwa. Lokhu kubizwa ngokuthi ukwelashwa kwe-neoadjuvant (preoperative).

Izinhlobo ezilandelayo zokuhlinzwa zingasetshenziswa:

  • Ukukhishwa kwendawo yonke: Ukususwa kwesimila kanye nezicubu ezithile ezijwayelekile ezisizungezile.
  • Ukunqunywa: Ukuhlinzwa ukususa konke noma ingxenye yengalo noma umlenze onomdlavuza.
  • I-Lymphadenectomy: Ukususwa kwama-lymph node nomdlavuza.
  • Ukuhlinzwa kukaMohs: Inqubo yokuhlinza esetshenziselwa ukwelapha umdlavuza esikhunjeni. Izendlalelo ngazinye zezicubu zomdlavuza ziyasuswa futhi zihlolwe ngaphansi kwesibonakhulu esisodwa kuze kube yilapho zonke izicubu zomdlavuza zisusiwe. Lolu hlobo lokuhlinzwa lusetshenziselwa ukwelapha i-dermatofibrosarcoma protuberans.

Kubizwa nangokuthi ukuhlinzwa kwe-Mohs micrographic.

  • I-Hepatectomy: Ukuhlinzwa ukususa konke noma ingxenye yesibindi.

Ukuhlinzwa kwesibili kungadingeka ukuze:

  • Susa noma yimaphi amangqamuzana omdlavuza asele.
  • Hlola indawo ezungeze lapho kukhishwe khona isimila ukuthola amangqamuzana omdlavuza bese ususa izicubu eziningi uma kudingeka.

Uma umdlavuza usesibindi, kungenziwa ukufakelwa i-hepatectomy nokufakwa kwesibindi (isibindi siyasuswa kufakwe esinempilo esivela kumnikeli).

Ngemuva kokuthi udokotela awususe wonke umdlavuza ongabonakala ngesikhathi sokuhlinzwa, ezinye iziguli zinganikezwa i-chemotherapy noma i-radiation therapy ngemuva kokuhlinzwa ukubulala noma yimaphi amangqamuzana omdlavuza asele. Ukwelashwa okunikezwe ngemuva kokuhlinzwa, ukwehlisa ubungozi bokuthi umdlavuza uzobuya, kubizwa ngokuthi yi-adjuvant therapy.

Ukwelashwa ngemisebe

Ukwelashwa ngemisebe ukwelashwa komdlavuza okusebenzisa amandla amakhulu ama-x-ray noma ezinye izinhlobo zemisebe ukubulala amangqamuzana omdlavuza noma ukuwagcina ekukhuleni. Kunezinhlobo ezimbili zokwelashwa ngemisebe:

  • Ukwelashwa kwemisebe kwangaphandle kusebenzisa umshini ongaphandle komzimba ukuthumela imisebe ebheke emdlavuzeni. Izindlela ezithile zokunikeza ukwelashwa ngemisebe kungasiza ukugcina imisebe ekulimaleni izicubu ezinempilo eziseduze. Lolu hlobo lokwelashwa ngemisebe lungafaka okulandelayo:
  • Ukwelashwa kwemisebe yomzimba we-stereotactic: Ukwelashwa kwemisebe yomzimba we-stereotactic uhlobo lwezokwelapha ngemisebe yangaphandle. Kusetshenziswa imishini ekhethekile ukubeka isiguli endaweni efanayo ekwelashweni kwemisebe ngayinye. Kanye ngosuku izinsuku eziningana, umshini wemisebe uhlose umthamo omkhulu kunokwejwayelekile wemisebe ngqo esihlahleni. Ngokuba nesiguli endaweni efanayo ekwelashweni ngakunye, kunomonakalo omncane ezicutshini eziseduze ezinempilo. Le nqubo ibizwa nangokuthi i-stereotactic external-beam radiation therapy kanye ne-stereotaxic radiation therapy.
  • Ukwelashwa kwemisebe kwangaphakathi kusebenzisa into enemisebe eboshwe ngezinaliti, izimbewu, izintambo, noma ama-catheters abekwe ngqo noma eduze komdlavuza.

Ukuthi ukwelashwa ngemisebe kunikezwa ngaphambi noma ngemuva kokuhlinzwa ukukhipha umdlavuza kuya ngohlobo nesigaba somdlavuza owelaswayo, uma kukhona amangqamuzana omdlavuza ahlala ngemuva kokuhlinzwa, kanye nemiphumela emibi elindelekile yokwelashwa. Ukwelashwa kwemisebe yangaphandle neyangaphakathi kusetshenziselwa ukwelapha i-sarcoma yezicubu ezithambile zobuntwana.

Ukwelashwa ngamakhemikhali

I-Chemotherapy yindlela yokwelapha umdlavuza esebenzisa izidakamizwa ukuvimba ukukhula kwamangqamuzana omdlavuza, kungaba ngokubulala amaseli noma ngokuwavimba ekuhlukaniseni. Lapho i-chemotherapy ithathwa ngomlomo noma ifakwa emthanjeni noma emisipheni, izidakamizwa zingena egazini futhi zingafinyelela kumaseli womdlavuza emzimbeni wonke (systemic chemotherapy). Lapho i-chemotherapy ifakwa ngqo ku-cerebrospinal fluid, isitho, noma umgodi womzimba njengesisu, izidakamizwa zithinta kakhulu amangqamuzana omdlavuza kulezo zindawo (i-chemotherapy yesifunda). Inhlanganisela yokwelashwa ngamakhemikhali ukusetshenziswa kwezidakamizwa ezingaphezu kweyodwa ze-antiticancer

Indlela i-chemotherapy enikezwa ngayo incike ohlotsheni lwe-sarcoma ethambile yezicubu elashwa. Izinhlobo eziningi ze-sarcoma yezicubu ezithambile aziphenduli ekwelashweni ngamakhemikhali.

Bheka Izidakamizwa Ezivunyelwe I-Soft Tissue Sarcoma ukuthola eminye imininingwane.

Ukuqaphela

Ukuqaphelisisa kusibhekisisa isimo sesiguli ngaphandle kokunikeza ukwelashwa kuze kuvele izimpawu noma izimpawu noma ushintsho. Ukuqaphela kungenziwa lapho:

  • Ukususwa ngokuphelele kwesimila akunakwenzeka.
  • Azikho ezinye izindlela zokwelapha ezitholakalayo.
  • Isimila kungenzeka ukuthi singalimazi noma yiziphi izitho ezibalulekile.

Ukuqaphela kungasetshenziselwa ukwelapha uhlobo lwe-desmoid-fibromatosis, i-infantile fibrosarcoma, i-PEComa, noma i-epithelioid hemangioendothelioma.

Ukwelashwa okuhlosiwe

Ukwelashwa okuhlosiwe uhlobo lokwelashwa olusebenzisa izidakamizwa noma ezinye izinto ukuhlasela amangqamuzana omdlavuza. Izindlela zokwelashwa ezihlosiwe zivame ukudala ukulimala okuncane kumaseli ajwayelekile kunokwenza ngamakhemikhali noma ngemisebe.

  • Ama-Kinase inhibitors avimba i-enzyme ebizwa nge-kinase (uhlobo lweprotheyini). Kunezinhlobo ezahlukahlukene ze-kinases emzimbeni ezinezenzo ezihlukile.
  • Ama-ALK inhibitors angavimba umdlavuza ukuthi ukhule futhi usabalale. I-Crizotinib ingasetshenziselwa ukwelapha isimila esivuthayo se-myofibroblastic kanye ne-infantile fibrosarcoma.
  • Izimpawu zokuvimba zeTyrosine kinase inhibitors (TKIs) ezidingekayo ukuze izicubu zikhule. I-Imatinib isetshenziselwa ukwelapha i-dermatofibrosarcoma protuberans. I-Pazopanib ingasetshenziselwa ukwelapha i-desmoid-type fibromatosis, i-epithelioid hemangioendothelioma, nezinye izinhlobo ze-sarcoma yezicubu ezithambile eziphindaphindayo. I-Sorafenib ingasetshenziselwa ukwelapha uhlobo lwe-desmoid-fibromatosis kanye ne-epithelioid hemangioendothelioma. I-Sunitinib ingasetshenziselwa ukwelapha i-alveolar soft part sarcoma. I-Larotrectinib isetshenziselwa ukwelapha i-infantile fibrosarcoma. I-Ceritinib isetshenziselwa ukwelapha isimila esivuthayo se-myofibroblastic.
  • I-mTOR inhibitors wuhlobo lokwelashwa okuqondisiwe okumisa amaprotheni asiza amaseli ahlukane futhi aphile. Ama-mTOR inhibitors asetshenziselwa ukwelapha izicubu zeseli eziyindilinga eziphindaphindayo ezine-desmoplastic, i-PEComas, ne-epithelioid hemangioendothelioma futhi kusacutshungulwa ukwelapha isimila esibucayi somgogodla womgogodla. I-Sirolimus ne-temsirolimus yizinhlobo zokwelashwa kwe-mTOR inhibitor.

Izinhlobo ezintsha ze-tyrosine kinase inhibitors ziyafundwa njenge:

  • I-Entrectinib ye-infantile fibrosarcoma.
  • I-Trametinib ye-epithelioid hemangioendotheliomaoma.

Ezinye izinhlobo zokwelashwa okuhlosiwe ziyafundwa ekuhlolweni kwemitholampilo, kufaka phakathi okulandelayo:

  • Ama-angiogenesis inhibitors awuhlobo lwezokwelapha ezihlosiwe ezivimbela ukukhula kwemithambo yegazi emisha edingekayo ukuze izicubu zikhule. Ama-angiogenesis inhibitors, afana ne-cediranib, i-sunitinib, ne-thalidomide afundelwa ukwelapha i-alveolar soft part sarcoma ne-epithelioid hemangioendothelioma. I-Bevacizumab isetshenziselwa ukwelapha i-angiosarcoma.
  • I-Histone methyltransferase (HMT) inhibitors yimithi yokwelapha ehlosiwe esebenza ngaphakathi kwamangqamuzana omdlavuza futhi ivimbe amasiginali adingekayo ukuze izimila zikhule. Ama-HMT inhibitors, afana ne-tazemetostat, afundelwa ukwelashwa kwesimila esibi se-peripheral nerve sheath, i-epithelioid sarcoma, i-extraskeletal myxoid chondrosarcoma, ne-extrarenal (extracranial) rhabdoid tumor.
  • Ama-protein-inhibitors ashubisa ukushisa avimba amaprotheni athile avikela amangqamuzana ezimila futhi abasize bakhule. I-Ganetespib iyi-heat shock protein inhibitor efundwa ihlanganiswe ne-mTOR inhibitor sirolimus yamathumba amabi e-peripheral nerve sheath angenakususwa ngokuhlinzwa.
  • I-NOTCH pathway inhibitors uhlobo lwezokwelapha ezihlosiwe ezisebenza ngaphakathi kwamangqamuzana omdlavuza futhi zivimba amasiginali adingekayo ukuze izimila zikhule.

I-NOTCH pathway inhibitors ifundelwa ukwelashwa kwe-desmoid-type fibromatosis.

Bheka Izidakamizwa Ezivunyelwe I-Soft Tissue Sarcoma ukuthola eminye imininingwane.

I-Immunotherapy

I-Immunotherapy yimithi esebenzisa amasosha omzimba esiguli ukulwa nomdlavuza. Izinto ezenziwe ngumzimba noma ezenziwe elabhoratri zisetshenziselwa ukukhulisa, ukuqondisa, noma ukubuyisa izivikelo zemvelo zomzimba kumdlavuza. Lolu hlobo lwezokwelapha lubizwa nangokuthi i-biotherapy noma i-biologic therapy.

I-Interferon kanye ne-immune checkpoint inhibitor therapy yizinhlobo ze-immunotherapy.

  • I-Interferon iphazamisa ukuhlukaniswa kwamangqamuzana we-tumor futhi inganciphisa ukukhula kwe-tumor. Isetshenziselwa ukwelapha i-epithelioid hemangioendotheliomaoma.
  • Ukwelashwa kwe-immune checkpoint inhibitor therapy: Ezinye izinhlobo zamaseli omzimba, njengama-T cell, namanye amangqamuzana omdlavuza anamaprotheni athile, abizwa ngokuthi amaprotheni wokuhlola, ebusweni bawo agcina izimpendulo zomzimba zibheke. Lapho amangqamuzana omdlavuza enenqwaba yalawa maprotheni, ngeke ahlaselwe futhi abulawe ngamaseli we-T. Amasosha omzimba okuhlola izivikeli mzimba avimba lawa maprotheni futhi kwanda ikhono lamaseli T ukubulala amangqamuzana omdlavuza.

Kunezinhlobo ezimbili zokwelashwa kwama-immune checkpoint inhibitor therapy:

  • I-CTLA-4 inhibitor: I-CTLA-4 yiprotheni ebusweni bamaseli we-T asiza ukugcina izimpendulo zomzimba womzimba zihloliwe. Lapho i-CTLA-4 inamathela kwenye iphrotheni ebizwa nge-B7 esitokisini somdlavuza, iyamisa i-T cell ekubulaleni iseli lomdlavuza. I-CTLA-4 inhibitors inamathela ku-CTLA-4 futhi ivumela amaseli we-T ukuthi abulale amangqamuzana omdlavuza. I-Ipilimumab uhlobo lwe-CTLA-4 inhibitor efundelwa ukwelapha i-angiosarcoma.
Isithiyo sokuhlola izivikeli mzimba. Amaprotheni e-Checkpoint, afana ne-B7-1 / B7-2 kumaseli we-antigen-presenting (APC) kanye ne-CTLA-4 kuma-T cell, asiza ukugcina izimpendulo zomzimba womzimba zibheke. Lapho i-T-cell receptor (TCR) ibophezela kuma-antigen kanye namaprotheni amakhulu we-histocompatibility complex (MHC) ku-APC naku-CD28 ibopha ku-B7-1 / B7-2 ku-APC, i-T cell ingasebenza. Kodwa-ke, ukubopha kwe-B7-1 / B7-2 kuya ku-CTLA-4 kugcina amaseli we-T esesimweni sokungasebenzi ngakho-ke awakwazi ukubulala amangqamuzana e-tumor emzimbeni (iphaneli langakwesobunxele). Ukuvimba ukubopha kwe-B7-1 / B7-2 kuya ku-CTLA-4 nge-immune checkpoint inhibitor (anti-CTLA-4 antibody) kuvumela ama-T cell ukuthi asebenze futhi abulale amangqamuzana wesimila (iphaneli lesokudla).
  • I-PD-1 inhibitor: I-PD-1 yiprotheni ebusweni bamaseli we-T asiza ukugcina izimpendulo zomzimba womzimba zihloliwe. Lapho i-PD-1 inamathela kwenye iprotheni ebizwa nge-PDL-1 kuseli lomdlavuza, iyeka i-T cell ekubulaleni iseli lomdlavuza. Ama-PD-1 inhibitors anamathisela ku-PDL-1 futhi avumela amaseli we-T ukuthi abulale amangqamuzana omdlavuza. I-Pembrolizumab uhlobo lwe-PD-1 inhibitor esetshenziselwa ukwelapha i-sarcoma yezicubu ezithambile neziqhubekayo. I-Nivolumab uhlobo lwe-PD-1 inhibitor efundelwa ukwelapha i-angiosarcoma.
Isithiyo sokuhlola izivikeli mzimba. Amaprotheni wokuhlola, njenge-PD-L1 kumaseli we-tumor ne-PD-1 kumaseli we-T, asiza ukugcina izimpendulo ze-immune zihlolwe. Ukubopha kwe-PD-L1 kuya ku-PD-1 kugcina amaseli we-T ekubulaleni amangqamuzana omzimba emzimbeni (iphaneli engakwesobunxele). Ukuvimba ukubopha kwe-PD-L1 kuya ku-PD-1 nge-immune checkpoint inhibitor (i-anti-PD-L1 noma i-anti-PD-1) ivumela amaseli we-T ukuthi abulale amangqamuzana wesimila (iphaneli elifanele).

Olunye Ukwelashwa Kwezidakamizwa

Ukwelashwa kwe-steroid kunemiphumela yokulwa nezicubu ezivuthayo ze-myofibroblastic.

Ukwelashwa kweHormone ukwelashwa komdlavuza okususa ama-hormone noma kuvimbe isenzo sabo futhi kumise amangqamuzana omdlavuza ukuthi angakhuli. AmaHormone yizinto ezenziwe yizindlala emzimbeni futhi zisatshalaliswa egazini. Amanye ama-hormone angadala ukuthi umdlavuza othile ukhule. Uma ukuhlolwa kukhombisa ukuthi amangqamuzana omdlavuza anezindawo lapho ama-hormone anganamathisela khona (ama-receptors), izidakamizwa, ukuhlinzwa, noma ukwelashwa ngemisebe kusetshenziselwa ukunciphisa ukukhiqizwa kwamahomoni noma ukuwavimba ekusebenzeni. Ama-antiestrogens (izidakamizwa ezivimba i-estrogen), njenge-tamoxifen, zingasetshenziswa ukwelapha uhlobo lwe-desmoid-fibromatosis. I-Prasterone ifundelwa ukwelashwa kwe-synovial sarcoma.

Izidakamizwa ezingezona ukuvuvukala (ama-NSAID) yizidakamizwa (njenge-aspirin, ibuprofen, ne-naproxen) evame ukusetshenziselwa ukunciphisa umkhuhlane, ukuvuvukala, ubuhlungu nokubomvu. Ekwelapheni i-desmoid-type fibromatosis, i-NSAID ebizwa nge-sulindac ingasetshenziswa ukusiza ukuvimba ukukhula kwamangqamuzana omdlavuza.

Izinhlobo ezintsha zokwelashwa ziyahlolwa ezivivinyweni zokwelashwa.

Lesi sigaba esifingqiwe sichaza izindlela zokwelashwa ezicwaningwa ekuhlolweni kwemitholampilo. Kungenzeka kungasho yonke imishanguzo emisha efundwayo. Imininingwane mayelana nokuhlolwa kwemitholampilo iyatholakala kuwebhusayithi ye-NCI.

Ukwelashwa kwe-Gene

Ukwelashwa kwe-Gene kufundelwa i-synovial sarcoma yobuntwana ephinde yabuya, yasakazeka, noma ayikwazi ukususwa ngokuhlinzwa. Amanye ama-T cell wesiguli (uhlobo lwengqamuzana yegazi elimhlophe) ayasuswa bese izakhi zofuzo ezisemangqamuzaneni ziguqulwa elabhorethri (eyenziwe izakhi zofuzo) ukuze zihlasele amangqamuzana athile omdlavuza. Bese babuyiselwa esigulini ngokumnika.

Iziguli zingafuna ukucabanga ngokubamba iqhaza esivivinyweni somtholampilo.

Kwezinye iziguli, ukubamba iqhaza esivivinyweni somtholampilo kungaba yindlela engcono kakhulu yokwelashwa. Ukuhlolwa kwemitholampilo kuyingxenye yenqubo yocwaningo lomdlavuza. Ukuhlolwa komtholampilo kuyenziwa ukuthola ukuthi ngabe ukwelashwa komdlavuza okusha kuphephile futhi kuyasebenza yini noma kungcono kunokwelashwa okujwayelekile.

Eziningi zezindlela zokwelashwa ezijwayelekile zomdlavuza zisuselwa ekuhlolweni kokuqala komtholampilo. Iziguli ezibamba iqhaza esivivinyweni somtholampilo zingathola ukwelashwa okujwayelekile noma zibe phakathi kwabokuqala ukuthola ukwelashwa okusha.

Iziguli ezibamba iqhaza ezivivinyweni zokwelashwa zisiza nokwenza ngcono indlela umdlavuza ozophathwa ngayo ngokuzayo. Noma izilingo zomtholampilo zingaholeli ekwelashweni okusha okusebenzayo, zivame ukuphendula imibuzo ebalulekile futhi zisize ukuqhubela phambili ucwaningo.

Iziguli zingangena ezivivinyweni zokwelashwa ngaphambi, ngesikhathi, noma ngemuva kokuqala ukwelashwa kwazo komdlavuza.

Ezinye izivivinyo zokwelashwa zifaka kuphela iziguli ezingakatholi ukwelashwa. Ezinye izilingo zivivinya ukwelashwa kweziguli ezinomdlavuza ongakabi ngcono. Kukhona nokuhlolwa komtholampilo okuhlola izindlela ezintsha zokunqanda umdlavuza ukuthi ungaphindi (ubuye) noma kunciphise imiphumela emibi yokwelashwa komdlavuza.

Ukuhlolwa kwemitholampilo kuyenzeka ezingxenyeni eziningi zezwe. Imininingwane mayelana nokuhlolwa kwemitholampilo esekelwa yi-NCI ingatholakala ekhasini lewebhu le-NCI lokuhlolwa kokuhlolwa kwemitholampilo. Izivivinyo zomtholampilo ezisekelwa ezinye izinhlangano zingatholakala kuwebhusayithi yeClinicalTrials.gov.

Ukuhlolwa kokulandelela kungadingeka.

Olunye uvivinyo olwenziwe ukuxilonga umdlavuza noma ukuthola isigaba somdlavuza lungaphindwa. Olunye uvivinyo luzophindwa ukuze kubonakale ukuthi umuthi usebenza kanjani. Izinqumo mayelana nokuthi uqhubeke, ushintshe, noma umise ukwelashwa zingasuselwa emiphumeleni yalezi zivivinyo.

Ezinye zezivivinyo zizoqhubeka ukwenziwa ngezikhathi ezithile ngemuva kokuphela kokwelashwa. Imiphumela yalezi zivivinyo ingakhombisa ukuthi ngabe isimo sengane yakho sishintshile noma uma umdlavuza ubuyile (buyela emuva). Lezi zivivinyo kwesinye isikhathi zibizwa ngokuthi izivivinyo zokulandelela noma ukuhlolwa.

Izinketho Zokwelashwa Kwethishu Yesikhumba Esithambile Esitholakele

Kulesi Sigaba

  • Izicubu Zezicubu Zamafutha
  • I-Liposarcoma
  • Izicubu Zamathambo Nezindlala
  • I-extraceletelet mesenchymal chondrosarcoma
  • I-osteosarcoma eyengeziwe
  • Izicubu Zamathambo Ezihlanganisayo (Ezixhumanisayo)
  • Uhlobo lwe-Desmoid-fibromatosis
  • Ama-protuberans eDermatofibrosarcoma
  • Isimila esivuthayo se-myofibroblastic
  • I-Fibrosarcoma
  • I-Myxofibrosarcoma
  • I-low-grade fibromyxoid sarcoma
  • Ukushaya i-epithelioid fibrosarcoma
  • Izicubu Zemisipha Yamathambo
  • I-Rhabdomyosarcoma
  • Izimila Zemisipha Ebushelelezi
  • I-Leiomyosarcoma
  • Okuthiwa yi-Fibrohistiocytic Tumors
  • Isimila se-Plexiform fibrohistiocytic
  • Izimila Zomgogodla
  • Isigaxa semithambo esibuhlungu esibuhlungu
  • Isimila esibi seTriton
  • I-Ectomesenchymoma
  • Izimila ze-Pericytic (Perivascular)
  • I-hemangiopericytoma yezinsana
  • I-myofibromatosis yezinsana
  • Izidumbu Zomsuka Wamaseli Ongaziwa (indawo lapho kuqale khona isimila akaziwa)
  • I-Synovial sarcoma
  • I-Epithelioid sarcoma
  • Ingxenye ethambile ye-alveolar sarcoma
  • Sula i-cell sarcoma yezicubu ezithambile
  • I-extrondeletal myxoid chondrosarcoma
  • I-Extraskeletal Ewing sarcoma
  • I-Desmoplastic small cell cell isimila
  • Isigaxa se-rhabdoid esingeziwe (i-extracranial)
  • Izicubu zamangqamuzana e-Perivascular epithelioid (PEComas)
  • I-Sarcoma engahlukanisiwe / engahlonziwe
  • I-pleomorphic sarcoma / i-histiocytoma enobungozi (ibanga eliphakeme)
  • Izimila Zesitsha Segazi
  • I-Epithelioid hemangioendotheliomaoma
  • I-Angiosarcoma yezicubu ezithambile
  • I-Metastatic Childhood Tissue Sarcoma

Ngemininingwane mayelana nokwelashwa okubalwe ngezansi, bheka isigaba se-Treatment Option Overview section

Izicubu Zezicubu Zamafutha

I-Liposarcoma

Ukwelashwa kwe-liposarcoma kungafaka okulandelayo:

  • Ukuhlinzwa ukususa ngokuphelele isimila. Uma umdlavuza ungasuswanga ngokuphelele, kungenziwa ukuhlinzwa kwesibili.
  • I-Chemotherapy yokunciphisa isimila, kulandele ukuhlinzwa.
  • Ukwelashwa ngemisebe ngaphambi kokuhlinzwa noma ngemuva kokuhlinzwa.

Izicubu Zamathambo Nezindlala

I-extraceletelet mesenchymal chondrosarcoma

Ukwelashwa kwe-extraceletelet mesenchymal chondrosarcoma kungafaka okulandelayo:

  • Ukuhlinzwa ukususa ngokuphelele isimila. Ukwelashwa ngemisebe kunganikezwa ngaphambi kanye / noma ngemuva kokuhlinzwa.
  • I-Chemotherapy yalandelwa ukuhlinzwa. I-Chemotherapy enokwelashwa ngemisebe noma ngaphandle kwayo inikezwa ngemuva kokuhlinzwa.

I-osteosarcoma eyengeziwe

Ukwelashwa kwe-osteosarcoma engaphandle kungafaka okulandelayo:

  • Ukuhlinzwa ukukhipha ngokuphelele isimila, kulandelwe ukwelashwa ngamakhemikhali.

Bona isifinyezo se- ku-Osteosarcoma naseMalignant Fibrous Histiocytoma ye-Bone Treatment ukuthola eminye imininingwane mayelana nokwelashwa kwe-osteosarcoma.

Izicubu Zamathambo Ezihlanganisayo (Ezixhumanisayo)

Uhlobo lwe-Desmoid-fibromatosis

Ukwelashwa kwe-desmoid-type fibromatosis kungafaka okulandelayo:

  • Ukuhlinzwa ukususa ngokuphelele isimila.
  • Ukuqaphela, kwezimila ezingasuswanga ngokuphelele ngokuhlinzwa noma eziphinde zabuya (zibuya) futhi ezingeke zilimaze noma yiziphi izitho ezibalulekile.
  • I-Chemotherapy yezimila ezingasuswa ngokuphelele ngokuhlinzwa noma eziphinde zabuya.
  • Ukwelashwa okuhlosiwe (i-sorafenib noma i-pazopanib).
  • Ukwelashwa kwe-nonsteroidal anti-inflammatory drug (NSAID).
  • Ukwelashwa kwezidakamizwa i-antiestrogen.
  • Ukwelashwa ngemisebe.
  • Isivivinyo somtholampilo sokwelapha okuhlosiwe nge-NOTCH pathway inhibitor.

Ama-protuberans eDermatofibrosarcoma

Ukwelashwa kwe-dermatofibrosarcoma protuberans kungafaka okulandelayo:

  • Ukuhlinzwa ukukhipha ngokuphelele isimila lapho kungenzeka. Lokhu kungafaka ukuhlinzwa kukaMohs.
  • Ukwelashwa ngemisebe ngaphambi kokuhlinzwa noma ngemuva kokuhlinzwa.
  • Ukwelashwa ngemisebe nokwelashwa okuqondisiwe (imatinib) uma isimila singeke sisuswe noma sibuya.

Isimila esivuthayo se-myofibroblastic

Ukwelashwa kwesimila esivuthayo se-myofibroblastic kungafaka okulandelayo:

  • Ukuhlinzwa ukukhipha ngokuphelele isimila lapho kungenzeka.
  • Ukwelashwa ngamakhemikhali.
  • Ukwelashwa kwama-steroid.
  • Ukwelashwa kwe-nonsteroidal anti-inflammatory drug (NSAID).
  • Ukwelashwa okuhlosiwe (i-crizotinib ne-ceritinib).

I-Fibrosarcoma

I-fibrosarcoma yezinsana

Ukwelashwa kwe-infile fibrosarcoma kungafaka okulandelayo:

  • Ukuhlinzwa ukukhipha isimila lapho kungenzeka, kulandelwe ukubonwa.
  • Ukuhlinzwa kwalandelwa ukwelashwa ngamakhemikhali.
  • I-Chemotherapy yokunciphisa isimila, kulandele ukuhlinzwa.
  • Ukwelashwa okuhlosiwe (i-crizotinib ne-larotrectinib).
  • Isivivinyo somtholampilo esihlola isampula lesimila sesiguli ukuthola izinguquko ezithile zofuzo. Uhlobo lokwelashwa okuqondisiwe oluzonikezwa isiguli kuncike ohlotsheni lokushintsha kofuzo.
  • Isivivinyo somtholampilo sokwelapha okuhlosiwe (i-larotrectinib noma i-entrectinib).

I-fibrosarcoma yabantu abadala

Ukwelashwa kwe-fibrosarcoma yabantu abadala kungafaka okulandelayo:

  • Ukuhlinzwa ukukhipha ngokuphelele isimila lapho kungenzeka.

I-Myxofibrosarcoma

Ukwelashwa kwe-myxofibrosarcoma kungafaka okulandelayo:

  • Ukuhlinzwa ukususa ngokuphelele isimila.

I-low-grade fibromyxoid sarcoma

Ukwelashwa kwe-fibromyxoid sarcoma esezingeni eliphansi kungafaka okulandelayo:

  • Ukuhlinzwa ukususa ngokuphelele isimila.

Ukushaya i-epithelioid fibrosarcoma

Ukwelashwa kwe-sclerosing epithelioid fibrosarcoma kungafaka okulandelayo:

  • Ukuhlinzwa ukususa ngokuphelele isimila.

Izicubu Zemisipha Yamathambo

I-Rhabdomyosarcoma

Bona isifinyezo se- ku-Childhood Rhabdomyosarcoma Treatment.

Izimila Zemisipha Ebushelelezi

I-Leiomyosarcoma

Ukwelashwa kwe-leiomyosarcoma kungafaka okulandelayo:

  • Ukwelashwa ngamakhemikhali.

Okuthiwa yi-Fibrohistiocytic Tumors

Isimila se-Plexiform fibrohistiocytic

Ukwelashwa kwesimila se-plexiform fibrohistiocytic kungafaka okulandelayo:

  • Ukuhlinzwa ukususa ngokuphelele isimila.

Izimila Zomgogodla

Isigaxa semithambo esibuhlungu esibuhlungu

Ukwelashwa kwesimila semfucumfucu eyingozi yomgogodla kungafaka okulandelayo:

  • Ukuhlinzwa ukukhipha ngokuphelele isimila lapho kungenzeka.
  • Ukwelashwa ngemisebe ngaphambi kokuhlinzwa noma ngemuva kokuhlinzwa.
  • I-Chemotherapy, yamathumba angenakususwa ngokuhlinzwa.
  • Isivivinyo somtholampilo esihlola isampula lesimila sesiguli ukuthola izinguquko ezithile zofuzo. Uhlobo lokwelashwa okuqondisiwe oluzonikezwa isiguli kuncike ohlotsheni lokushintsha kofuzo.
  • Isivivinyo somtholampilo sokwelapha okuhlosiwe (i-ganetespib noma i-sirolimus), yamathumba angenakususwa ngokuhlinzwa.
  • Isivivinyo somtholampilo sokwelapha okuhlosiwe (tazemetostat).

Akucaci ukuthi ukunikeza imishanguzo ngemisebe noma i-chemotherapy ngemuva kokuhlinzwa kuthuthukisa impendulo yesimila ekwelashweni.

Isimila esibi seTriton

Izimila zeMalignant Triton zingaphathwa ngokufana ne-rhabdomyosarcomas futhi zifaka ukuhlinzwa, i-chemotherapy, noma i-radiation therapy. Akucaci ukuthi ukunikeza imishanguzo ngemisebe noma i-chemotherapy kuyayithuthukisa yini impendulo yesimila ekwelashweni.

I-Ectomesenchymoma

Ukwelashwa kwe-ectomesenchymoma kungafaka okulandelayo:

  • Ukuhlinzwa.
  • Ukwelashwa ngamakhemikhali.
  • Ukwelashwa ngemisebe.

Izimila ze-Pericytic (Perivascular)

I-hemangiopericytoma yezinsana

Ukwelashwa kwe-hemangiopericytoma yezinsana kungafaka okulandelayo:

  • Ukwelashwa ngamakhemikhali.

I-myofibromatosis yezinsana

Ukwelashwa kwe-myofibromatosis yezinsana kungafaka okulandelayo:

Inhlanganisela chemotherapy.

Izidumbu Zomsuka Wamaseli Ongaziwa (indawo lapho kuqale khona isimila akaziwa)

I-Synovial sarcoma

Ukwelashwa kwe-synovial sarcoma kungafaka okulandelayo:

  • Ukuhlinzwa. Ukwelashwa ngemisebe kanye / noma i-chemotherapy kunganikezwa ngaphambi noma ngemuva kokuhlinzwa.
  • Ukwelashwa ngamakhemikhali.
  • Ukwelashwa kwemisebe ye-stereotactic yamathumba asabalale aya emaphashini.
  • Isivivinyo somtholampilo sokwelashwa kofuzo.
  • Isivivinyo somtholampilo sokwelapha i-hormone.

I-Epithelioid sarcoma

Ukwelashwa kwe-epithelioid sarcoma kungafaka okulandelayo:

  • Ukuhlinzwa ukukhipha isimila lapho kungenzeka.
  • Ukwelashwa ngamakhemikhali.
  • Ukwelashwa ngemisebe ngaphambi kokuhlinzwa noma ngemuva kokuhlinzwa.
  • Isivivinyo somtholampilo sokwelapha okuhlosiwe (tazemetostat).

Ingxenye ethambile ye-alveolar sarcoma

Ukwelashwa kwe-alveolar soft part sarcoma kungafaka okulandelayo:

  • Ukuhlinzwa ukukhipha ngokuphelele isimila lapho kungenzeka.
  • Ukwelashwa ngemisebe ngaphambi kokuhlinzwa noma ngemuva kokuhlinzwa, uma isimila singeke sisuswe ngokuphelele ngokuhlinzwa.
  • Ukwelashwa okubhekiswe (sunitinib).
  • Isivivinyo somtholampilo sokwelapha okubhekiswe kukho (i-cediranib noma i-sunitinib) yezingane.

Sula i-cell sarcoma yezicubu ezithambile

Ukwelashwa kwe-cell sarcoma ecacile yezicubu ezithambile kungafaka okulandelayo:

  • Ukuhlinzwa ukukhipha isimila lapho kungenzeka.
  • Ukwelashwa ngemisebe ngaphambi kokuhlinzwa noma ngemuva kokuhlinzwa.

I-extrondeletal myxoid chondrosarcoma

Ukwelashwa kwe-extraceletelet myxoid chondrosarcoma kungafaka okulandelayo:

  • Ukuhlinzwa ukukhipha isimila lapho kungenzeka.
  • Ukwelashwa ngemisebe.
  • Isivivinyo somtholampilo sokwelapha okuhlosiwe (tazemetostat).

I-Extraskeletal Ewing sarcoma

Bona isifinyezo se- ku-Ewing Sarcoma Treatment.

I-Desmoplastic small cell cell isimila

Akukho ukwelashwa okujwayelekile kwe-desmoplastic small cell cell tumor. Ukwelashwa kungafaka okulandelayo:

  • Ukuhlinzwa ukukhipha ngokuphelele isimila lapho kungenzeka.
  • I-Chemotherapy yalandelwa ukuhlinzwa.
  • Ukwelashwa ngemisebe.
  • I-Chemotherapy kanye nokwelashwa okuhlosiwe (i-temsirolimus), yamathumba aphindaphindiwe.

Isigaxa se-rhabdoid esingeziwe (i-extracranial)

Ukwelashwa kwesisu se-rhabdoid esengeziwe (okungaphezulu) (i-extracranial) kungafaka okulandelayo:

  • Ukuhlinzwa ukukhipha isimila lapho kungenzeka.
  • Ukwelashwa ngamakhemikhali.
  • Ukwelashwa ngemisebe.
  • Isivivinyo somtholampilo esihlola isampula lesimila sesiguli ukuthola izinguquko ezithile zofuzo. Uhlobo lokwelashwa okuqondisiwe oluzonikezwa isiguli kuncike ohlotsheni lokushintsha kofuzo.
  • Isivivinyo somtholampilo sokwelapha okuhlosiwe (tazemetostat).

Izicubu zamangqamuzana e-Perivascular epithelioid (PEComas)

Ukwelashwa kwama-perivascular epithelioid cell tumors kungafaka okulandelayo:

  • Ukuhlinzwa ukukhipha isimila.
  • Ukuqaphela okulandelwa ukuhlinzwa.
  • Ukwelashwa okuhlosiwe (i-sirolimus), yamathumba anezinguquko ezithile zofuzo futhi angeke asuswe ngokuhlinzwa.

I-Sarcoma engahlukanisiwe / engahlonziwe

I-pleomorphic sarcoma / i-histiocytoma enobungozi (ibanga eliphakeme)

Akukho ukwelashwa okujwayelekile kwalezi zicubu.

Bona isifinyezo se- ku-Osteosarcoma naseMalignant Fibrous Histiocytoma Yokwelashwa Kwamathambo ukuze uthole imininingwane mayelana nokwelashwa kwe-histiocytoma yamathambo amabi.

Izimila Zesitsha Segazi

I-Epithelioid hemangioendotheliomaoma

Ukwelashwa kwe-epithelioid hemangioendothelioma kungafaka okulandelayo:

  • Ukuqaphela.
  • Ukuhlinzwa ukukhipha isimila lapho kungenzeka.
  • I-Immunotherapy (i-interferon) kanye nokwelashwa okuhlosiwe (i-thalidomide, i-sorafenib, i-pazopanib, i-sirolimus) yamathumba okungenzeka asabalale.
  • Ukwelashwa ngamakhemikhali.
  • Ukufakelwa okuphelele kwe-hepatectomy nesibindi lapho isimila sisesibindi.
  • Isivivinyo somtholampilo sokwelapha okuhlosiwe (trametinib).

I-Angiosarcoma yezicubu ezithambile

Ukwelashwa kwe-angiosarcoma kungafaka okulandelayo:

  • Ukuhlinzwa ukususa ngokuphelele isimila.
  • Inhlanganisela yokuhlinzwa, i-chemotherapy, kanye ne-radiation therapy yama-angiosarcomas asabalale.
  • Ukwelashwa okuhlosiwe (i-bevacizumab) ne-chemotherapy yama-angiosarcomas aqala njengama-hemangiomas amancane.
  • Isivivinyo somtholampilo se-chemotherapy noma ngaphandle kwokwelapha okuhlosiwe (pazopanib).
  • Isivivinyo somtholampilo se-immunotherapy (nivolumab ne-ipilimumab).

I-Metastatic Childhood Tissue Sarcoma

Ukwelashwa kwe-sarcoma yezicubu ezithambile ebuntwaneni eye yasakazeka kwezinye izingxenye zomzimba lapho kutholakala ukuxilongwa kungafaka okulandelayo:

  • Ukwelashwa ngamakhemikhali nokwelashwa ngemisebe. Ukuhlinzwa kungenziwa ukuze kususwe izicubu ezisabalalele emaphashini.
  • Ukwelashwa kwemisebe yomzimba we-stereotactic yamathumba asabalalele emaphashini.

Ukuze uthole ukwelashwa kwezinhlobo ezithile zesimila, bheka isigaba senketho yokwelashwa kwesigaba se-Childhood Tissue Sarcoma.

Izinketho zokwelashwa zeTissue Sarcoma yezicubu eziphindaphindayo nezithuthukayo zobuntwana

Ukwelashwa kwe-sarcoma yezicubu ezithambile noma eziqhubekayo zobuntwana kungafaka okulandelayo:

  • Ukuhlinzwa kususa umdlavuza obuye ubuyele lapho uqale wakha khona noma osusabalele emaphashini.
  • Ukuhlinzwa okulandelwa ukwelashwa ngemisebe kwangaphandle noma kwangaphakathi, uma ukwelashwa ngemisebe kunganikezwa.
  • Ukuhlinzwa ukususa ingalo noma umlenze ngomdlavuza, uma ngabe ukwelashwa ngemisebe bese kunikezwe.
  • Ukuhlinzwa nge-chemotherapy noma ngaphandle kwe-synovial sarcoma ephindaphindiwe.
  • Ukwelashwa ngamakhemikhali.
  • Ukwelashwa okuhlosiwe (pazopanib).
  • I-Immunotherapy (pembrolizumab).
  • Ukwelashwa kwemisebe yomzimba ye-stereotactic yomdlavuza osudlulele kwezinye izingxenye zomzimba, ikakhulukazi iphaphu.
  • Isivivinyo somtholampilo sohlobo olusha lwe-chemotherapy regimen noma ngaphandle kwokwelapha okuhlosiwe (pazopanib).
  • Isivivinyo somtholampilo esihlola isampula lesimila sesiguli ukuthola izinguquko ezithile zofuzo. Uhlobo lokwelashwa okuqondisiwe oluzonikezwa isiguli kuncike ohlotsheni lokushintsha kofuzo.

Sebenzisa ukusesha kwethu kokuhlolwa komtholampilo ukuthola izivivinyo zomtholampilo ezisekelwa yi-NCI ezamukela iziguli. Ungasesha izivivinyo ngokususelwa ohlotsheni lomdlavuza, iminyaka yesiguli, nalapho kwenziwa khona izivivinyo. Imininingwane ejwayelekile mayelana nokuhlolwa kwemitholampilo iyatholakala.

Ukufunda Kabanzi Nge-Tissue Sarcoma Yezingane Ezithambile

Ngemininingwane engaphezulu evela ku-National Cancer Institute mayelana nengane ethambile yezicubu sarcoma, bheka okulandelayo:

  • Ikhasi lasekhaya le-Soft Tissue Sarcoma
  • I-Computed Tomography (CT) Iskena Nomdlavuza
  • Izidakamizwa zivunyelwe i-Soft Tissue Sarcoma
  • Izindlela Zokwelapha Zomdlavuza
  • I-MyPART - Inethiwekhi Yami Yezingane ne-Adult Rare Tumor Network

Ngemininingwane eminingi yomdlavuza wezingane nezinye izinsiza zomdlavuza ezijwayelekile, bheka okulandelayo:

  • Mayelana Nomdlavuza
  • Umdlavuza Wezingane
  • I-CureSearch ye-Cancer YezinganePhuma Ukuzikhulula
  • Imiphumela Emuva Yokwelapha Yomdlavuza Wezingane
  • Intsha kanye nabantu abadala abancane abaneCancer
  • Izingane Ezinomdlavuza: Umhlahlandlela Wabazali
  • Umdlavuza Ezinganeni Nasebusheni
  • Isiteji
  • Ukubhekana Nomdlavuza
  • Imibuzo Ongayibuza Udokotela Wakho mayelana Nomdlavuza
  • Kwabasindile Nabanakekeli
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