Rhabdomyosarcoma Embryonal: unyango, abantu abane

Rhabdomyosarcoma libhekisela uhlobo nee- - umhlaza kwizihlunu ezithambileyo, ithambo okanye kwizicwili. Ithumba kakhulu ukuba kwenzeka izihlunu eziqhotyoshelwe amathambo. Rhabdomyosarcoma - yobubi evela esihlunwini skeletal. Baqala kuyo nayiphi na inxalenye enye lomzimba okanye kwiindawo ezininzi.

Microscopically rhabdomyosarcoma abadala na uhlobo iiseli evuthelweyo okanye elirhangqiweyo kwelilingeneyo engaqhelekanga. uphawu ngaphezulu eli ithumba of ebuntwaneni.

iintlobo rhabdomyosarcoma

Rhabdomyosarcoma lobume yangaphakathi zohlulwa ngokwendidi:

1. iiseli rhabdomyosarcoma Embryonal ibandakanya ngeenxa kunye fusiform kukho icytoplasm wakhe uphando lubonisa striations longitudinal okanye enqamlezileyo. Olu hlobo rhabdomyosarcoma zenzeka rhoqo. Livela rhabdomyosarcoma embryonal abantwana esakhula. Le ise entanyeni okanye entloko, phezu zangasese, kwi nasopharynx. Uvakalelo yamathumba kuthomalaliso imitha, kodwa agule ibuye ngokukhawuleza.

2. Alveolar rhabdomyosarcoma sakhiwa iqela leeseli ngeenxa kunye okweqanda, leyo singqongwe septa yethishyu nokuqina. Ngokuba lo hlobo yamathumba luphawulwa yimbonakalo kwi imilenze okanye iingalo, isisu, ibele, ezimpundu okanye amalungu obuni. Kaninzi ingafunyanwa kwi abafikisayo kunye nabantu abadala phezu imilenze. Abantu abane ulihlwempu.

3. Ukwakhiwa rhabdomyosarcoma pleomorphic - yokuluka-ezimilise, iribhoni-ezifana, inkwenkwezi-ezimile iiseli polymorphic ye nethumba leyo angavela iinyawo okanye izandla zabantu. Njengomgaqo, ukukhula rhabdomyosarcoma kwenzeka ngokukhawuleza kwaye ukuvise intlungu nokuphazamiseka ezisebenzayo. Oku kwenzeka mithambo kuphela. Ngokufuthi zikhule ngokusebenzisa nesikhumba egcwele izilonda, ukwakha exophytic, ithumba ukopha.

ubungozi

Phakathi iimeko zokusingqongileyo bafumanisa ezinjalo, ezandisa amathuba isenzeko thumba. Oku kwahlukile ezininzi zomhlaza rhabdomyosarcoma.

Ngenxa yokuba akukho ngqo eyaziwa oonobangela zophuhliso ithumba asinakho, ukunika ingcebiso ngokuba uyaku- lasungulwa. Noko ke, simele sikhumbule ukuba unyango olwaneleyo kwezigulane rhabdomyosarcoma amaxesha sinyangeke ngokupheleleyo.

Imiba yobungozi yimbonakalo rhabdomyosarcoma osandul izifo zemfuza sizimisele ezifana:

1. Li-Fraumeni syndrome sisifo ezinqabileyo apho ilifa phawu, umonakalo gene xa ulahlekile umzali omnye ukuya kumaza eliphezulu kwavela iintlobo ezahlukeneyo ithumba. Njengelifa kulo ngezifo wadlulisela ingakumbi ithumba reactions zeethishu xa kakhulu kwandisa amathuba okuba yokuphuhlisa yamathumba esemncinane (engama-30).

2. Neurofibromatosis Uhlobo lokuqala - esi sifo ilifa ixhaphakileyo ebangela inkangeleko ukuthambekela yobubi.

3. Syndrome Beckwith-Wiedemann - ngezifo, nto leyo luphawulwa macrosomia, macroglossia kunye omphalocele. Kwakhona kubonakala iziphene ebantwaneni zodonga phambi kwe esiswini, Ithemba, hypoglycaemia lweentsana, lo divergence kwemisipha rectus.

4. Costello syndrome - isifo esinqabileyo, nto leyo zibandakanya malformations iziphene: abazizidalwa ngokukhula, iimpawu zobuso erhabaxa, utshintsho isikhumba.

5. syndrome Noonan kaThixo - yokugula uphawuleka omfutshane ngesithomo apho izinto ezinxaxhileyo kwaye kuphuhliso somatic. Oko kunokuthi kwenzeke entsatsheni okanye nayiphi na amalungu ayo.

Abantwana baba ubunzima zokuzalwa kunye nokukhula, ube ngaphezu kwabanye, umngcipheko rhabdomyosarcoma embryonal. Noko ke, njengoko umthetho, oonobangela rhabdomyosarcoma embryonal awaziwa. A umqondiso esi sifo ukuvela ithumba iya ngakumbi nangakumbi.

iimpawu

Symptomatology isifo somhlaza uzimisele. Kufuneka ngoko nangoko udibane nogqirha xa abantwana baye zidweliswe iimpawu zomhlaza:

• nokudumba okanye ukudumba, ukwanda ngobukhulu okanye abadlulayo, maxa wambi buhlungu;
• amehlo equmbile;
• yintloko ebuhlungu;
• ubunzima kunye defecation kunye ukuchama;
• igazi nomchamo;
• ukopha isikali, impumlo kunye nomqala.

ekuxilongweni

Ukuza kuthi ga ngoku, liqulunqe iindlela ukuze kugqitywe rhabdomyosarcoma de iimpawu zayo lokuqala nezimanyumnyezi.

Iimpawu zokuqala zomhlaza - iindumbisa wengingqi okanye induration, oko kunokubangela iingxaki ekuqaleni okanye intlungu. Le uphawu le uphawu rhabdomyosarcoma, iingalo, imilenze kunye umntla.

Ukuba Ithumba yenzelwe kwindawo esiswini okanye zangasese kunokubangela iintlungu esiswini, ugabhe okanye ukuqhina. It rhabdomyosarcoma embryonal bendawo retroperitoneal. Okunye Kunqabile, abasaphuhlayo iphecana biliary, rhabdomyosarcoma ibangela senyongo.

Ukuba kukho urhano yale ithumba, kuyimfuneko ukuba aqhube biopsy, nto leyo eya cacisa uhlobo rhabdomyosarcoma isuka.

Kwiimeko ezininzi, kuvela rhabdomyosarcoma kwiindawo apho ngokulula elibhaqiweyo, umzekelo, lweHo- ngeempumlo okanye ngaphandle lweliso lakho. Ukuba emehlweni equmbile, okanye netuwa ngeempumlo abonakale, kufuneka ngoko nangoko udibane nogqirha ithumba ekukrokrelwa ukuba unesifo kusekwangoko yophuhliso lwayo. Kwimeko apho rhabdomyosarcoma livela phezu komhlaba emzimbeni, oko ngokulula ibhaqe ngaphandle uphando olunzulu. Oku kubizwa ngokuba yi-rhabdomyosarcoma embryonal inyama ithambile.

Ngowe-30% ezigulane kunye rhabdomyosarcoma embryonal babe yinto kusekwangoko xa lungasuswa ngokupheleleyo. Kodwa ke uninzi lwezigulane ezineenkcukacha zoviwo ityhila metastases ezincinane, unyango lwe elifuna phambi kusetyenziswe amachiza.

rhabdomyosarcoma Embryonal, apho ibekwe kwindawo betyhalarha rhoqo ziveliswa ebantwaneni abancinane bafumana abazali bomntwana xa uhlamba. Ukuba yamathumba kwenzeka womchamo, bevakalise nzima ukuchama okanye ukumpompoza kwegazi - iimpawu angena kubethwa ngoyaba.

isigaba ithumba

1. Inyathelo lokuqala. Ithumba inokuba nayiphi na ubukhulu, kodwa okwangoku kwalutyhutyha kwindawo nkovu kwenkqubo kwaye ibekwe kwenye yeendawo prognostically kakuhle umzimba:

- emehlweni okanye indawo periocular;

- Intloko nentamo (ngaphandle izitho wobuchopho indawo umnqonqo);

- enyongweni imibhobho kunye nenyongo isinyi;

- kule isibeleko okanye etyumke ubudoda.

Iindawo ngaphandle kuluhlu, zijongwa iindawo ezimbi.

2. Inqanaba lesibini. Ukudumba yenye imimandla ukungaphatheki kakuhle (imimandla apho zilahlekile evumayo). ubukhulu Ithumba ngaphantsi kwe-5 cm kwaye asiqhenqethanga ukuya kwiindawo nkovu sele yenzekile.

3. Inqanaba lesithathu. Ithumba ibekwe kwindawo kakubi, kwaye uzalisekise enye yezi meko zilandelayo:

- ubungakanani ithumba ngaphantsi kwe-5 cm, yaye isasazeka phezu kwiindawo nkovu elandelayo;

- nethumba mkhulu kuno-5 cm, kwaye kukho i-risk yokuba siza kwanda ukuya nkovu node elikufutshane.

4. Isigaba sesine. ubukhulu Ithumba kuba nantoni na, yaye yanwenwela kwiindawo nkovu elandelayo. bekusoloko ukusasazeka umhlaza kwiindawo ezikude umzimba apho.

amaqela ingozi

Eyabelwe iqela umngcipheko isigulane imisela amathuba ukuphinda rhabdomyosarcoma. Nawuphi na umntwana ofumana unyango evela rhabdomyosarcoma embryonal, kufuneka bafumane ichemotherapy ezongezelelweyo ukuze zibe nokwenzeka ngaphantsi kwe ithumba ukuphinda. View iziyobisi anticancer, idosi Inani leeseshoni - konke oku kumiselwa umngcipheko nawuphi na umntwana ekubhekiselwe - phantsi, eliphakathi okanye eliphezulu.

iinketho zonyango

Izigulane rhabdomyosarcoma embryonal zinokunyangwa ngeendlela ezahlukeneyo.
Unyango ibandakanya yamathumba amabi, iindlela ezahlukileyo leyo kuthathwa ukuba zemveli. Ezinye iindlela phantsi yolingo lwezonyango. Ulingo lwezonyango aze asebenzise ukuphucula iindlela ezikhoyo zokuyisebenza okanye ukunyusa ubungakanani idatha iindlela ezintsha zokunyanga izigulane rhabdomyosarcoma. Ukuba ngenxa yokuba ulingo lonyango, kubonakala ukuba iindlela ezintsha zonyango kunabanye yemveli, unyango entsha ingena kudidi lwe zemveli.

Ekubeni yamathumba ebulalayo zikholisa ukuvela kwiindawo ezahlukeneyo zomzimba, kusebenza inani elikhulu unyango. Unyango lwe rhabdomyosarcoma kubantwana kweliso somdlavuza ezingabantwana.

Phakathi iindlela zonyango, kukho abathile oko kunokubangela iziphumo ezingalindelekanga, naxa wadlula unyango ukusukela ixesha elide.

utyando

kwendlela yotyando, okanye ukususwa nethumba, lisetyenziswa kwi kunyango rhabdomyosarcoma. Lo msebenzi ibizwa "Ukusikwa ekuhlaleni ngokubanzi". Le iswitch ukususa yinxalenye thumba kunye izihlunu ezikufutshane kunye ezalathelwe uphuhliso nkovu ukuba nayo kuchaphazela rhabdomyosarcoma. Unyango ngamanye amaxesha kukuba ukwenza imisebenzi emininzi. Isigqibo sokuqeshwa ukusebenza kunye nohlobo yayo ixhomekeke ezi zinto zilandelayo:

- beka indawo yokuqala nethumba;

- nayiphi na imisebenzi umntwana lomzimba abachatshazelwa ithumba;

- impendulo Ithumba ku radiotherapy okanye ichemotherapy, enokusetyenziselwa qho ephambili.

Uninzi ukususwa labantwana ngayo yonke ivolyum thumba yi uqhaqho akwenzeki.

Embryonal rhabdomyosarcoma kwizihlunu ezithambileyo lidla ukuba abunjwe kwiindawo ezahlukeneyo emzimbeni, nganye kuzo ifuna ukusebenza yalo. ungenelelo kuhlinzwa kuyinto kubonisiwe kwimeko eveze rhabdomyosarcoma okanye amehlo, ukuba biopsy ukuqinisekisile uxilongo. Phambi kokuba umsebenzi linokukwenziwa ukuba ichemotherapy okanye radiotherapy ukunciphisa nangezilonda ezinkulu, zibe nolusebenza, ukuba lula umsebenzi lokususa umhlaza.

Nokuba ugqirha ayikwazi ukususa yonke ithumba, izigulane ekupheleni umsebenzi kufuneka phantsi ichemotherapy ukucinezela iiseli zomhlaza ukuba bahlale bephila. Kunye chemotherapy, kusetyenziswa unyango radiation. Unyango ejoliswe ekunciphiseni ubungozi ukwenzeka sifo kuthiwa unyango adjuvant.

therapy radiation

Nale hlobo lonyango kusetyenziswa X-ray okanye enye kwemitha. therapy Imitha ngqo kwi into, ukuba bambulale iiseli ithumba okanye ayeke ekukhuleni kwayo. isicelo sayo amayeza baye bafumana iindlela ezimbini ukuya radiotherapy - lwangaphandle nangaphakathi.

Yangaphandle isebenzisa umthombo radiation ngaphandle komzimba womntu ngenxa irradiating nethumba. therapy radiation lwangaphakathi okanye brachytherapy irradiates ithumba ekupapashweni izinto radioactive kwi kufutshane ngaphakathi emzimbeni womntu. Isetyenziswa ukunyanga umhlaza isinyi, intloko kunye nentamo, yesinye, womntu obhinqileyo.

Uhlobo unyango radiation kunye nedosi emiselwe ubudala bomntwana kunye nohlobo ithumba, kwasendaweni yayo ekuqaleni kodwa ke ubukho ithumba intsalela kunye nezinga kobhaliso ithumba nodes nkovu elikufutshane emva kotyando.

chemotherapy

Olu hlobo unyango lomhlaza esebenzisa amayeza ukunqanda ukukhula iiseli zomhlaza. iziyobisi chemotherapy inceda ukubulala ezi seli okanye ukuthintela ukwahlukana kwabo. A amachiza chemotherapy ezicwangcisiweyo zithathwa ngomlomo okanye lesitofu yegazi okanye intramuscularly. Ukufika igazi, iziyobisi zichaphazela iiseli umhlaza wonke umzimba. Xa iziyobisi chemotherapy akwingingqi lesitofu ngqo amalungu kunye nemingxuma nomzimba, kwakunye kulwelo fluid. Kolimo chemotherapy isoloko isetyenziswa, nto leyo unyango esetyenziswa anti-umhlaza iziyobisi ngaphezu kwesinye. indlela chemotherapy kukhethiwe ngokuxhomekeke kuhlobo kunye ibakala uphuhliso neoplasm.

Chemotherapy iboniswe umntwana wonke ubani liphathwa ukuba rhabdomyosarcoma. It ikuvumela ukuba zokunciphisa ukuphinda sifo. Ukhetho amayeza, ithamo kunye nenani iinkqubo ezichazwe iqela lomngcipheko rhabdomyosarcoma.

unyango olutsha

iintlobo ezintsha rhabdomyosarcoma unyango, edlula uvavanyo:

• ngeedosi amakhulu nechemotherapy ngokudibene ne ekuqhubeni iseli stem esinye. Le ndlela yenza kube lula ukuba alungiselele ukutshintshwa iiseli hematopoietic ukuba kutshatyalaliswa ngexesha unyango lomhlaza. Iiseli ubuntwana asuswe phambi kokuba unyango lwe lomongo wethambo okanye igazi umguli kunye yagcinwa. Ekupheleni ichemotherapy raskonserviruyut ilondolozwe iiseli stem waza wabuyela kwisigulana yi ukumnika. Ngaloo iiseli igazi ukuntshula.
• Immunotherapy. Xa olu hlobo unyango ukulwa somhlaza usebenzisa amajoni omzimba yesigulana. amachiza ezilawulwa ngayo ukuphucula okanye ukubuyisela eziphumelela zendalo nomhlaza. Olu nyango kuthiwa biotherapy yamathumba.
• therapy ekujoliswe kuzo. Olu hlobo unyango sibhekiswe Ukudlulela. unyango ekujoliswe isekelwe phezu ukusetyenziswa omzimba athile yekeyeke kwiiseli umhlaza. Ezi zithinteli iyafana izilwa eqhelekileyo umzimba womntu. Ngaloo bamele ichiza eliphezulu-zobugcisa. Ngenxa therapy ekujoliswe kulo unyango "chemistry" inokufumaneka ukuhlasela iiseli umhlaza kuphela ngaphandle kokuzenzakalisa iiseli esempilweni. Xa rhabdomyosarcoma in angiogenesis inhibitors zisetyenziswa nonyango target. La machiza kuthintela ukuyilwa imithambo yegazi ngamathumba. Oku kukhokelela ekubeni ukuba unciphise thumba nezingxi ekhulayo. inhibitors Angiogenesis kunye antibodies monoclonal - iindidi zee-arhente zizilingo lwezonyango lokulawula rhabdomyosarcoma osandul.