ʻO ka maʻi maʻi akā hoʻomau a hiki ke hōʻeha loa i ka ʻāina, nā ʻōpū desmoid, a i ʻole fibromatosis aggressive, he mau maʻi maʻi kakaikahi e ulu ana mai nā ʻiʻo a me nā envelopes ʻiʻo (aponeuroses). ʻO ka hoʻomohala ʻole ʻana, hiki iā lākou ke kumu o ka ʻeha a me ka ʻeha hana nui. He paʻakikī ka hoʻokele a koi i ke komo ʻana o kahi hui loea multidisciplinary.

Definition

ʻO nā maʻi maʻi Desmoid a i ʻole fibromatosis aggressive he mau maʻi maʻi i hana ʻia me nā sela fibrous e like me nā cell maʻamau i loko o ka ʻiʻo fibrous i kapa ʻia he fibroblasts. No ka mahele o na ma'i ma'i ho'ohui ("soft" tissue tumors), ua ulu mai lakou mai na 'i'o a i 'ole nā ​​envelopes muscle (aponeuroses).

He mau maʻi maʻi maʻi kēia - ʻaʻole ia ke kumu o ka metastases - akā no ka hoʻomohala ʻike ʻole ʻia, ka mea i hōʻike pinepine ʻia i ka hoʻouka kaua ʻana i ka ʻāina a me ka hoʻi hou ʻana inā paha e ulu liʻiliʻi kekahi a i ʻole e hoʻihoʻi koke.

Hiki iā lākou ke ala mai ma nā wahi a pau o ke kino. Hiki i nā ʻaoʻao papa ke hele i nā lālā a me ka paia o ka ʻōpū, akā ʻo ka ʻāʻī a me ke poʻo (i nā keiki liʻiliʻi) a i ʻole ka ʻāʻī hiki ke noho. Aia kekahi ʻano hohonu o nā maʻi maʻi desmoid (intra-abdominal localization).

Nā kumu

ʻAʻole maopopo i ke kumu o nā maʻi maʻi desmoid, akā ua manaʻo ʻia he multifactorial, me ke komo ʻana o nā mea hormonal a me nā genetic.

ʻO ka ʻeha a i ʻole ka hōʻeha ʻana (scars) kekahi o nā kumu o ko lākou helehelena, a me ka hānau ʻana (ma ke kiʻekiʻe o ka paia o ka ʻōpū).

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Hōʻike nā hoʻokolohua kiʻi i ka loaʻa ʻana o kahi infiltrating mass e ulu ana i ka wā. Hoʻokumu nui ʻia ka ʻike ma ka CT (computed tomography a i ʻole CT) no nā maʻi maʻi intra-abdominal a i ʻole MRI (Magnetic Resonance Imaging) no nā maʻi koko ʻē aʻe.

Pono ka biopsy e hōʻoia i ka hōʻoia. No ka pale ʻana i ka pilikia o ka huikau, pono e hana ʻia ka histological analysis (no ka nānā ʻana ma lalo o kahi microscope) e ke kauka loea i ka pathology me ka ʻike i kēia mau maʻi koko.

Hiki ke hana ʻia nā hoʻāʻo genetic i ka ʻike ʻana i nā mutations hiki ke hana.

Pili ka poʻe

Hoʻopilikia ka maʻi maʻi Desmoid i ka poʻe ʻōpio, e piʻi ana ma kahi o 30 mau makahiki. Hoʻopilikia pū ʻia nā keiki, ʻoi aku hoʻi i ka wā ʻōpio. 

He maʻi maʻi ʻaʻole ia (0,03% o nā maʻi maʻi āpau), e ʻike ʻia me ke alapine i manaʻo ʻia i kēlā me kēia makahiki ma 2 a 4 mau hihia hou i kēlā me kēia miliona mau kānaka.

Nā mea pilikino

I loko o nā ʻohana i hoʻopilikia ʻia e ka ʻohana adenomatous polyposis, kahi maʻi hoʻoilina i ʻike ʻia e ka noho ʻana o nā colon multiples, ʻoi aku ka nui o ka pilikia o ka hoʻomohala ʻana i kahi maʻi maʻi desmoid ma mua o ka heluna lehulehu a ua manaʻo ʻia aia ma kahi o 10 a 15%. Hoʻopili ʻia me ka hoʻololi ʻana i kahi gene i kapa ʻia ʻo APC (tumor suppressor gene), pili i kēia maʻi.

Eia nō naʻe, ʻike pinepine ʻia ka hapa nui o nā maʻi fibromatosis aggressive (me ka ʻole o ka hoʻoilina). Ma kahi o 85% o kēia mau hihia hiki ʻole ke hoʻololi ʻia, pili ʻia ka hoʻololi ʻana o ka maʻi tumora me nā hoʻololi ʻole o ka gene. CTNNB1, ke kumu o ka hoʻololi ʻana o kahi pūmua e pili ana i ka mālama ʻana i ka hoʻonui ʻana o ka maʻi maʻi i kapa ʻia ʻo beta-catenin.

Kauiho

Hoʻokumu nā maʻi maʻi Desmoid i ka pehu i ʻike ʻia ma ka palpation ma ke ʻano he paʻa, mobile, i kekahi manawa nui loa "nā pōpō" e pili pinepine ana i nā hale kūlohelohe kokoke.

eha

ʻAʻole ʻeha ka ʻōpū ma kāna iho akā hiki ke hōʻeha i ka ʻiʻo koʻikoʻi, ka ʻōpū a i ʻole ka ʻeha aʻalolo ma muli o kona wahi.

Nā genes hana

ʻO ka hoʻoemi ʻana i nā ʻiʻo e pili ana i hiki ke hana i nā ʻano like ʻole. ʻO ka paʻi ʻana o nā aʻalolo, no ka laʻana, ke kumu o ka emi ʻana o ka neʻe ʻana o kahi lālā. Hoʻopilikia nā ʻano hohonu i nā kīʻaha koko, ka ʻōpū a i ʻole ka ʻōnaehana urinary, etc.

Hiki ke nalowale ka hana o ke kino i pili.

He kuni kekahi ma'i ma'i desmoid.

ʻAʻohe hoʻolālā lapaʻau maʻamau a hoʻoholo ʻia i kēlā me kēia hihia e kahi hui multidisciplinary loea.

Hiki ke ʻeha nā maʻi maʻi desmoid paʻa a pono e mālama i ka ʻeha. 

ʻO ka nānā kiaʻi

Hoʻomaʻamaʻa mua ʻia, ua haʻalele ʻia ke ʻoki ʻana i kēia manawa no ke ʻano conservative e pili ana i ka nānā pono ʻana i ka ulu ʻana o ka maʻi maʻi ma mua o ka hoʻokau ʻana i kahi lāʻau kaumaha i kekahi manawa ʻaʻole pono.

ʻOiai ke kūpaʻa ka ʻōpū, pono paha ka mālama ʻana i ka ʻeha.

ʻoki kino

ʻOi aku ka maikaʻi o ka wehe ʻana i ka ʻōpū desmoid inā hiki a hiki i ka hoʻonui ʻia ʻana o ka ʻōpū me ka ʻole o ka lilo ʻana o ka hana nui (e laʻa, ka ʻoki ʻana o kahi lālā).

ʻO ke kelepona

Hiki ke hoʻohana ʻia e hoʻāʻo e hoʻihoʻi a hoʻopaʻa paha i ka maʻi maʻi desmoid, i ka wā e piʻi ai, o ka hoʻi hou ʻana a i ʻole e hōʻemi i ka pilikia o ka hoʻi hou ʻana ma hope o ke ʻoki ʻana. Ma muli o kona hopena maikaʻi ʻole i ka ulu ʻana o nā kānaka a hoʻohana liʻiliʻi ʻia i nā keiki. 

Nā hōʻailona lapaʻau

Loaʻa i nā molekala like ʻole ka maikaʻi i hoʻokumu ʻia a hoʻohana ʻia hoʻokahi a i hui pū ʻia. ʻO ka tamoxifen, kahi lāʻau anti-estrogen ikaika e hoʻohana ʻia i ka wā e maʻa ai ka ʻōpū i kēia hormone wahine, i nā lāʻau anti-inflammatory non-steroidal, i nā ʻano like ʻole o ka chemotherapy (methotrexate, vinblastine / vinorelbine, pegylated liposomal doxorubicin) a i ʻole. ʻO nā lāʻau lapaʻau molecular i hoʻopaʻa ʻia e pale i ka ulu ʻana o ka maʻi tumora (imatinib, sorafenib), hāʻawi ʻia ma ke ʻano he papa.

ʻO nā hana ʻē aʻe

• Hoʻohana ʻia ka Cryotherapy ma lalo o ka anesthesia maʻamau e luku ai i nā maʻi maʻi ma ka hoʻokuʻu ʻana iā lākou i loko

  – 80 ° C.

• Hoʻokomo ʻia ka lāʻau lapaʻau i hoʻokaʻawale ʻia i ka hoʻokomo ʻana i ka chemotherapy kiʻekiʻe i loko o ka lālā i hoʻopilikia wale ʻia.

Hāʻawi ʻia kēia mau kaʻina hana ma kekahi mau kikowaena loea ma Farani.

Evolution

Ma kahi o 70% o nā hihia, ʻike ʻia ka hoʻi hou ʻana o ka maʻi maʻi. ʻAʻole pili ka prognosis koʻikoʻi, koe wale nō ka pilikia o ka ʻoki ʻana, ʻo ia hoʻi no nā ʻōpū o ka ʻōpū.