Hōʻike ʻia ka sarcoma o Ewing e ka ulu ʻana o kahi maʻi maʻi maʻi i loko o nā iwi a me nā ʻiʻo palupalu. Loaʻa i kēia ʻōpū ke ʻano me ka hiki ke metastatic kiʻekiʻe. ʻIke pinepine ʻia ka laha ʻana o nā cell tumor ma ke kino i kēia pathology.

He maʻi kakaʻikahi e pili ana i nā keiki. He 1/312 ka nui o na keiki malalo o 500 makahiki.

ʻO ka pūʻulu makahiki i hoʻopilikia nui ʻia e ka ulu ʻana o kēia ʻano maʻi maʻi maʻi ma waena o 5 a 30 mau makahiki, a ʻoi aku ka nui o ka maʻi ma waena o 12 a me 18 mau makahiki. (3)

ʻO nā hōʻike lapaʻau e pili ana i ka ʻeha a me ka pehu ma kahi o ka ʻōpū.

ʻO nā wahi o nā pūnana maʻi maʻi e like me ka sarcoma o Ewing he nui: nā wāwae, nā lima, nā wāwae, nā lima, ka umauma, ke pelvis, ka iwi poʻo, ka iwi kuamoʻo, a pēlā aku.

Ua kapa ʻia kēia sarcoma Ewing: ʻo ka ʻōpū neuroectodermal peripheral mua. (1)

ʻO nā hoʻokolohua lapaʻau e ʻae i ka hiki ke ʻike i ka maʻi a hoʻoholo i kona pae o ka holomua. ʻO ka hoʻokolohua pili pinepine ʻia he biopsy.

Hiki i nā mea kiko'ī a me nā kūlana ke hoʻopili i ka wānana o ka maʻi i kahi kumuhana i hoʻopilikia ʻia. (1)

ʻO kēia mau mea e pili ana i ka hoʻolaha ʻana o nā sela maʻi maʻi i nā māmā wale nō, ʻoi aku ka maikaʻi o ka wānana, a i ʻole ka ulu ʻana o nā ʻano metastatic i nā wahi ʻē aʻe o ke kino. I ka hihia hope, ʻoi aku ka maikaʻi o ka prognosis.

Eia kekahi, ʻo ka nui o ka ʻōpū a me ka makahiki o ka mea i hoʻopilikia ʻia he kuleana koʻikoʻi i ka prognosis koʻikoʻi. ʻOiaʻiʻo, inā piʻi ka nui o ka maʻi ma mua o 8 cm, ʻoi aku ka hopohopo o ka prognosis. No ka makahiki, ʻoi aku ka maikaʻi o ka maʻi o ka pathology, ʻoi aku ka maikaʻi o ka prognosis no ka mea maʻi. (4)

ʻO ka sarcoma o Ewing kekahi o nā ʻano nui ʻekolu o ka maʻi maʻi iwi mua me ka chondrosarcoma a me ka osteosarcoma. (2)

ʻO nā hōʻailona i pili pinepine ʻia me ka sarcoma o Ewing, ʻo ia ka ʻeha a me ka pehu ʻana i nā iwi i hoʻopilikia ʻia a me nā ʻiʻo palupalu.

 ʻO nā hōʻike maʻi maʻi e hiki mai ana i ka ulu ʻana o ia sarcoma: (1)

• ʻeha a / a pehu paha ma nā lima, nā wāwae, ka umauma, ke kua a i ʻole ke pelvis;
• ka hiki ʻana o nā "puʻu" ma kēia mau ʻāpana like o ke kino;
• ka hele ʻana o ke kuni no ke kumu ʻole;
• haʻi iwi no ke kumu kumu ʻole.

ʻO nā hōʻailona pili naʻe e pili ana i kahi o ka ʻōpū a me kona koʻikoʻi ma ke ʻano o ka hoʻomohala ʻana.

ʻO ka ʻeha i ʻike ʻia e ka mea maʻi me kēia pathology e hoʻonui pinepine i ka manawa.

 Hiki ke ʻike ʻia nā hōʻailona maʻamau ʻē aʻe, e like me: (2)

• he kuni kiekie a mau;
• ʻoʻoleʻa o ka mākala;
• poho kaumaha nui.

Eia naʻe, ʻaʻohe hōʻailona o ka mea maʻi me ka sarcoma o Ewing. Ma kēia ʻano, hiki ke ulu ka ʻōpū me ka ʻole o kahi hōʻike lapaʻau kūikawā a pēlā e hoʻopilikia ai i ka iwi a i ʻole ka ʻiʻo palupalu me ka ʻike ʻole ʻia. ʻOi aku ka nui o ka pilikia o ka haʻihaʻi ʻana i ka hihia hope. (2)

ʻOiai ʻo Ewing's sarcoma kahi ʻano o ka maʻi kanesa, ʻaʻole ʻike nui ʻia e pili ana i ke kumu maoli o kona ulu ʻana.

Ua waiho ʻia naʻe he kuhiakau e pili ana i ke kumu o kona ulu ʻana. ʻOiaʻiʻo, pili ka sarcoma o Ewing i nā keiki ma luna o 5 mau makahiki a me nā ʻōpio. Ma kēia ʻano, ua hoʻāla ʻia ka pilina ma waena o ka ulu wikiwiki ʻana o ka iwi ma kēia ʻāpana o ke kanaka a me ka ulu ʻana o ka sarcoma o Ewing.

ʻO ka wā o ka wā ʻōpio i nā keiki a me nā ʻōpio e ʻoi aku ka maʻalahi o nā iwi a me nā ʻiʻo palupalu i ka ulu ʻana o kahi ʻōpū.

Ua hōʻike pū ʻia ka noiʻi ʻana ʻekolu manawa o ke keiki i hānau ʻia me ka hernia umbilical e hoʻomohala i ka sarcoma o Ewing. (2)

Ma waho aʻe o kēia mau kuhiakau i ʻōlelo ʻia ma luna nei, ua waiho ʻia ke kumu e pili ana i ka hiki ʻana mai o kahi unuhi genetic. Aia kēia unuhi i ka gene EWSRI (22q12.2). Ua loaʻa kahi unuhi t (11; 22) (q24; q12) i loko o kēia moʻo hoihoi ma kahi o 90% o nā maʻi maʻi. Eia kekahi, nui nā ʻano genetic i kumu o ka noiʻi ʻepekema, e pili ana i nā genes ERG, ETV1, FLI1 a me NR4A3. (3)

Mai ka manaʻo i kahi o ke kumu maoli o ka pathology, a hiki i kēia lā, ʻaʻole i ʻike maikaʻi ʻia, ʻo nā kumu pilikia kekahi.

Eia kekahi, e like me nā hopena o nā noiʻi ʻepekema, ʻoi aku ka nui o ke keiki i hānau ʻia me ka hernia umbilical.

Eia kekahi, ma ka pae genetic, ʻo ka hiki ʻana o nā translocations i loko o ka EWSRI gene (22q12.2) a i ʻole nā ​​ʻano ʻano genetic i loko o nā genes ERG, ETV1, FLI1 a me NR4A3, ke kumu o nā kumu pilikia hou aʻe no ka hoʻomohala ʻana i ka maʻi. .

Hoʻokumu ʻia ka ʻike o ka sarcoma o Ewing ma kahi hōʻailona ʻokoʻa ma o ka loaʻa ʻana o nā hōʻailona ʻano i ka mea maʻi.

Ma hope o ka nānā ʻana o ke kauka i nā wahi ʻeha a pehu, ua kuhikuhi pinepine ʻia kahi x-ray. Hiki ke hoʻohana ʻia nā ʻōnaehana kiʻi lapaʻau ʻē aʻe, e like me: Magnetic Reasoning Imaging (MRI) a i ʻole scans.

Hiki ke hana ʻia kahi biopsy iwi no ka hōʻoia ʻana a i ʻole ka ʻike. No kēia, lawe ʻia kahi laʻana o ka iwi iwi a nānā ʻia ma lalo o kahi microscope. Hiki ke hana ʻia kēia ʻano hana diagnostic ma hope o ka anesthesia āpau a i ʻole ka ʻāina.

Pono e hoʻokō koke ʻia ka ʻike o ka maʻi i hiki ke hana koke ʻia ka mālama ʻana a no laila ʻoi aku ka maikaʻi o ka wānana.

 ʻO ka lāʻau lapaʻau no ka sarcoma o Ewing e like me ka mālama maʻamau no nā maʻi maʻi ʻē aʻe: (2)

• ʻO ka ʻokiʻoki he ala maikaʻi ia e mālama ai i kēia ʻano sarcoma. Eia nō naʻe, pili ka hana ʻokiʻoki i ka nui o ka ʻōpū, kona wahi a me kona pae o ka laha. ʻO ka pahuhopu o ka ʻoki ʻana, ʻo ia ke hoʻololi i ka ʻāpana o ka iwi a i ʻole ka ʻiʻo palupalu i hōʻino ʻia e ka ʻōpū. No kēia, hiki ke hoʻohana ʻia kahi prosthesis metala a i ʻole ka iwi iwi i ka pani ʻana o ka wahi i hoʻopilikia ʻia. I nā hihia koʻikoʻi, pono ka ʻoki ʻana i nā lālā e pale i ka hoʻi hou ʻana o ka maʻi kanesa;
• ka chemotherapy, hoʻohana mau ʻia ma hope o ka ʻoki ʻana e hōʻemi i ka ʻōpū a maʻalahi i ka ho'ōla.
• ʻO ka radiotherapy, hoʻohana pinepine ʻia ma hope o ka chemotherapy, ma mua a ma hope paha o ke ʻoki ʻana e hōʻemi i ka nui o ka ʻōpū a pale i ka pilikia o ka hoʻi hou ʻana.