Ewing's sarcoma imadziwika ndi kukula kwa chotupa choyipa m'mafupa ndi minofu yofewa. Chotupa ichi chimakhala ndi mphamvu zambiri za metastatic. Kapena kufalikira kwa maselo otupa m'thupi lonse nthawi zambiri kumadziwika ndi matenda awa.

Ndi matenda osowa kwambiri omwe amakhudza kwambiri ana. Chiwopsezo chake ndi ana 1/312 osakwana zaka 500.

Anthu amsinkhu omwe amakhudzidwa kwambiri ndi kukula kwa chotupa ichi ndi azaka zapakati pa 5 ndi 30, ndipo nthawi zambiri amakhala pakati pa 12 ndi 18. (3)

The kugwirizana matenda mawonetseredwe ululu ndi kutupa pa malo chotupacho.

Malo omwe ma cell a chotupa omwe amadziwika ndi Ewing's sarcoma ndi angapo: miyendo, mikono, mapazi, manja, chifuwa, pelvis, chigaza, msana, ndi zina zambiri.

Ewing sarcoma iyi imatchedwanso: chotupa choyambirira cha peripheral neuroectodermal. (1)

Kuyeza kwachipatala kumalola kuti matendawa athe kudziwa komanso kudziwa momwe akupita patsogolo. Kufufuza komwe kumachitika kawirikawiri ndi biopsy.

Zinthu zinazake ndi mikhalidwe ingakhudze momwe matendawa amakhalira pamutu womwe wakhudzidwa. (1)

Zinthuzi zikuphatikiza makamaka kufalikira kwa maselo otupa kupita m'mapapo okha, zomwe zimawoneka bwino kwambiri, kapena kukula kwa ma metastatic kumadera ena athupi. Pamapeto pake, matendawa amakhala ovuta.

Kuonjezera apo, kukula kwa chotupacho ndi zaka za munthu wokhudzidwayo zimakhala ndi gawo lofunikira pa chidziwitso chofunikira. Zowonadi, ngati kukula kwa chotupacho kumakwera kuposa masentimita 8, matendawa amakhala odetsa nkhawa. Ponena za msinkhu, matenda a matendawa akamayambika, zimakhala bwino kuti wodwalayo adziwe bwino. (4)

Ewing's sarcoma ndi imodzi mwa mitundu itatu yayikulu ya khansa ya m'mafupa komanso chondrosarcoma ndi osteosarcoma. (2)

Zizindikiro zomwe zimagwirizanitsidwa ndi Ewing's sarcoma ndi ululu wowoneka ndi kutupa m'mafupa okhudzidwa ndi minofu yofewa.

 Zizindikiro zotsatirazi zitha kukhala zoyambira pakukula kwa sarcoma yotere: (1)

• kupweteka ndi / kapena kutupa m'manja, miyendo, chifuwa, msana kapena chiuno;
• kukhalapo kwa "ziphuphu" pazigawo zomwezo za thupi;
• kukhalapo kwa malungo popanda chifukwa chenicheni;
• kuthyoka kwa mafupa popanda chifukwa chenicheni.

Zizindikiro zogwirizana komabe zimadalira malo a chotupacho komanso kufunika kwake pankhani ya chitukuko.

Ululu womwe wodwala wodwala matendawa amamva nthawi zambiri umakula pakapita nthawi.

 Zina, zizindikiro zocheperako zimatha kuwoneka, monga: (2)

• kutentha kwakukulu komanso kosalekeza;
• kuuma kwa minofu;
• kwambiri kuwonda.

Komabe, wodwala Ewing's sarcoma sangakhale ndi zizindikiro zilizonse. M'lingaliro limeneli, chotupacho chimatha kukula popanda kuwonetseredwa kwachipatala ndipo motero zimakhudza fupa kapena minofu yofewa popanda kuwoneka. Chiwopsezo cha fracture ndichofunika kwambiri pamapeto pake. (2)

Monga Ewing's sarcoma ndi mtundu wa khansa, zimadziwika pang'ono za chiyambi chake.

Komabe, lingaliro linaperekedwa mokhudzana ndi zomwe zidayambitsa kukula kwake. Zowonadi, Ewing's sarcoma imakhudza makamaka ana opitilira zaka 5 ndi achinyamata. M'lingaliro limeneli, kuthekera kwa kugwirizana pakati pa kukula kwa mafupa mofulumira m'gulu ili la munthu ndi chitukuko cha Ewing's sarcoma chakwezedwa.

Nthawi ya kutha msinkhu kwa ana ndi achinyamata imapangitsa mafupa ndi minofu yofewa kukhala pachiopsezo cha kukula kwa chotupa.

Kafukufuku wasonyezanso kuti mwana wobadwa ndi umbilical chophukacho ali ndi mwayi wochuluka kuwirikiza katatu kudwala Ewing's sarcoma. (2)

Pamwamba pa malingaliro awa omwe tawatchulawa, chiyambi cha kukhalapo kwa chibadwa cha translocation chaperekedwanso patsogolo. Kusamutsa kumeneku kumakhudza jini ya EWSRI (22q12.2). Kusuntha kwa A t (11; 22) (q24; q12) mkati mwa jini yosangalatsayi kunapezeka pafupifupi 90% ya zotupa. Kuonjezera apo, mitundu yambiri ya majini yakhala ikufufuzidwa ndi sayansi, kuphatikizapo majeremusi a ERG, ETV1, FLI1 ndi NR4A3. (3)

Kuchokera pakuwona komwe chiyambi chenicheni cha matendawa ndi, mpaka lero, chomwe sichikudziwika bwino, zifukwa zowopsa nazonso.

Komanso, malinga ndi zotsatira za maphunziro a sayansi, mwana wobadwa ndi umbilical chophukacho akhoza kuwirikiza katatu kukhala ndi mtundu wa khansa.

Kuonjezera apo, pamtundu wa chibadwa, kukhalapo kwa translocation mkati mwa jini la EWSRI (22q12.2) kapena mitundu yosiyanasiyana ya majini mu ERG, ETV1, FLI1 ndi NR4A3 jini, zikhoza kukhala zowonjezera zowonjezera zowonjezera matendawa. .

Kuzindikira kwa Ewing's sarcoma kumatengera kuzindikirika kosiyana mwa kukhalapo kwa zizindikiro za wodwalayo.

Kutsatira kuwunika kwa dokotala za madera opweteka ndi kutupa, x-ray nthawi zambiri imayikidwa. Njira zina zojambula zamankhwala zitha kugwiritsidwanso ntchito, monga: Magnetic Reasoning Imaging (MRI) kapena masikelo.

Mafupa amathanso kuchitidwa kuti atsimikizire kapena ayi. Pachifukwa ichi, chitsanzo cha mafupa amatengedwa ndikuwunikidwa pa microscope. Njira zodziwira matendawa zitha kuchitidwa pambuyo pa opaleshoni yamba kapena yapafupi.

Kuzindikira kwa matendawa kuyenera kuchitidwa mwamsanga kuti kasamalidwe kachitidwe kachitidwe mwamsanga ndipo motero matendawa amakhala bwino.

 Chithandizo cha Ewing's sarcoma ndi chofanana ndi chithandizo chambiri cha khansa zina: (2)

• Opaleshoni ndi njira yabwino yochizira mtundu uwu wa sarcoma. Komabe, chithandizo cha opaleshoni chimadalira kukula kwa chotupacho, malo ake komanso momwe amafalikira. Cholinga cha opaleshoni ndikusintha mbali ya fupa kapena minofu yofewa yomwe yawonongeka ndi chotupacho. Pachifukwa ichi, prosthesis yachitsulo kapena fupa la mafupa lingagwiritsidwe ntchito m'malo omwe akhudzidwa. Zikafika povuta kwambiri, kudula miyendo nthawi zina kumakhala kofunika kuti musayambirenso khansa;
• mankhwala a chemotherapy, omwe nthawi zambiri amagwiritsidwa ntchito pambuyo pa opaleshoni kuti achepetse chotupacho ndikuthandizira kuchira.
• radiotherapy, imagwiritsidwanso ntchito nthawi zambiri pambuyo pa chemotherapy, opaleshoni isanayambe kapena itatha kuti achepetse kukula kwa chotupacho ndikupewa chiopsezo choyambiranso.