Desmoid mamota

Desmoid mamota

Benign asi inodzokororwa uye iyo inogona kunge iri munharaunda zvakanyanya invasive, desmoid tumors, kana aggressive fibromatosis, mamota asingawanzo kubuda kubva munyama uye tsandanyama hamvuropu (aponeuroses). Kusimukira kusingatarisirwi, vanogona kuve kunobva marwadzo uye kushushikana kwakanyanya kwekushanda. Utungamiri hwakaoma uye hunoda kupindira kwechikwata chenyanzvi dzakawanda.

Chii chinonzi desmoid bundu?

tsananguro

Desmoid tumors kana aggressive fibromatosis mamota asingawanzo kugadzirwa nemaseru ane fibrous akafanana neakajairwa maseru mufibrous tissue inonzi fibroblasts. Iri muchikamu chezviputi zvinobatanidza ("zvinyoro" zviputi zviputi), zvinokura kubva mumhasuru kana mahamvuropu emhasuru (aponeuroses).

Aya mamota ane hutsinye - handiwo akonzeresa metastases - asi yekushanduka-shanduka kusingatarisike, iyo inowanzoratidza kuve inoparadza munharaunda uye inodzokororwa zvakanyanya kunyangwe mamwe akashanduka zvishoma kana kuti angangodzokera kumashure.

Vanogona kusimuka chero kupi mumuviri. Mafomu epamusoro anonyanya kusvika kumakumbo nemadziro emimba, asi mutsipa nemusoro (muvana vadiki) kana thorax inogonawo kuva chigaro. Kune zvakare mafomu akadzika e desmoid tumors (intra-abdominal localization).

Zvinokonzera

Mabviro emamota edemoid anoramba asinganzwisisike, asi anofungidzirwa kuve akawanda, nekubatanidzwa kwehomoni uye genetic factor.

Njodzi kana kuvhiyiwa kukuvadzwa (mavanga) anoita sechimwe chezvikonzero zvekuonekwa kwavo, pamwe nekuberekwa kwemwana (pamwero wemadziro emimba).

chirwere

Imaging bvunzo dzinoratidza kuvepo kwehuru inopinda iyo inokura nekufamba kwenguva. Kuongororwa kunonyanya kuenderana neCT (computed tomography kana CT) yemamota emukati-mudumbu kana MRI (Magnetic Resonance Imaging) yemamwe mapundu.

Biopsy inodiwa kusimbisa kuongororwa. Kubvisa njodzi yekuvhiringika, histological analysis (kuongorora pasi pemaikorosikopu) inofanira kuitwa nachiremba anoona nezvezvirwere zvine ruzivo rwemamota aya.

Genetic tests inogona kuitwa mukuwedzera kuona zvinogona kuchinja.

Vanhu vane hanya

Desmoid tumors inonyanya kubata vechidiki vechidiki, inosvika pazera remakore makumi matatu. Chirwere ichi chinonyanya kubata vanhukadzi. Vana vanofanirawo kukanganiswa, kunyanya vachiri kuyaruka. 

Ibundu risingawanzo (0,03% yemamota ese), rinoonekwa neakawanda anofungidzirwa gore rega rega kune maviri chete kusvika mana ezviitiko zvitsva pamiriyoni yevagari.

Zvinhu zvinotyisa

Mumhuri dzakabatwa nefamily adenomatous polyposis, chirwere chisingawanzogarwa nhaka chinoratidzwa nekuvapo kwecolon multiples, njodzi yekuva nebundu re desmoid yakakura kupfuura muhuwandu hwevanhu uye inofungidzirwa kunge iri pakati pe10 kusvika 15%. Inobatanidzwa nekuchinja kwejeni rinonzi APC (tumor suppressor gene), inobatanidzwa muchirwere ichi.

Nekudaro, ruzhinji rwezviitiko zveaggressive fibromatosis zvinowoneka zvishoma nezvishoma (pasina nhaka yekuzvarwa). Munenge 85% yezviitiko izvi zvisingatapurike, shanduko yebundu yemasero inobva yabatanidzwa netsaona shanduko yejini. CTNNB1, zvichikonzera kuchinjwa kweprotein iyo inobatanidzwa mukudzora kupararira kwebundu inonzi beta-catenin.

Zviratidzo zve desmoid tumors

Kuzvimba

Desmoid tumors inogadzira kuzvimba kunoonekwa palpation kunge yakasimba, nhare, dzimwe nguva yakakura kwazvo "mabhora" anowanzo kunamatira kune ari pedyo organic zvimiro.

marwadzo

Bundu racho harirwadziri roga asi rinogona kukonzera tsandanyama, mudumbu kana tsinga kurwadza zvichienderana nenzvimbo yaro.

Magene anoshanda

Iko kudzvanywa kunoitwa pamatishu akavakidzana kunogona kukonzera akasiyana mashandiro akashata. Kudzvinyirirwa kwemitsipa kunogona, semuenzaniso, kuva chikonzero chekuderera kwekufamba kwechikwata. Mafomu akadzika anokanganisa tsinga dzeropa, ura kana urinary system, nezvimwe.

Kurasikirwa kwekushanda kwenhengo inobatanidzwa kunogoneka.

Mamwe mapundu e desmoid anewo fivha.

Kurapa kwe desmoid tumors

Iko hakuna yakajairwa nzira yekurapa uye inosarudzwa pane imwe-ne-kesi hwaro nenyanzvi yemhando dzakasiyana-siyana timu.

Yakagadzikana desmoid tumors inogona kurwadza uye inoda kurapwa kwemarwadzo. 

Kuongorora kuri kuita

Kwaimboitwa, kuvhiyiwa kwave kusiiwa nekuda kwenzira inochengetedza iyo inosanganisira kunyatsoongorora kushanduka kwebundu risati raisa kurapwa kunorema dzimwe nguva kwaisazodikanwa.

Kunyange kana bundu rakadzikama, kurapwa kwemarwadzo kungave kuri madikanwa.

sejari

Kuvhiya kubviswa zvachose kwebundu redesmoid kunodiwa kana zvichibvira uye kuwedzera kwebundu kunobvumira pasina kukonzera kurasikirwa kukuru kwekushanda (semuenzaniso kudimburwa kwegumbo).

Radiotherapy

Inogona kushandiswa kuedza kuita kuti desmoid bundu ridzoke kana kudzikamisa, muchiitiko chekufambira mberi, rekudzoka kana kuderedza njodzi yekudzokazve mushure mekuvhiyiwa. Nekuda kwekukuvadza kwayo pakukura kwevanhu uye inoshandiswa zvishoma muvana. 

Zvinodhaka zvinodhaka

Mamorekuru akasiyana ane mamwe kana mashoma akanyatso kusimbiswa uye anoshandiswa ega kana musanganiswa. Kunyanya, tamoxifen, mushonga unoshanda unorwisa-estrogen unoshandiswa kana bundu richinzwa nehomoni iyi yechikadzi, kune isiri-steroidal anti-inflammatory drugs, kune marudzi akasiyana echemotherapy (methotrexate, vinblastine / vinorelbine, pegylated liposomal doxorubicin) kana kuti molecular therapeutics yakanangana nemishonga inovharisa bundu kukura (imatinib, sorafenib), yakapihwa semapiritsi.

Kumwe kurapa

  • Cryotherapy inoshandiswa pasi pe general anesthesia kuparadza mapundu nekuaomesa mukati

    -80 ° C.

  • Isolated Limb infusion inosanganisira kupinza high-dose chemotherapy munhengo yakabatwa chete.

Aya maitiro anongopihwa mune mashoma nyanzvi nzvimbo muFrance.

kushanduka

Munenge 70% yezviitiko, kudzoka kwenzvimbo yebundu kunoonekwa. Iyo yakakosha prognosis haina kubatanidzwa, kunze kwekunge kune yematambudziko ekuvhiyiwa, kunyanya kune abdominal tumors.

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