I-Benign kodwa ephindaphindayo futhi engahlasela kakhulu endaweni, ama-desmoid tumors, noma i-aggressive fibromatosis, amathumba ayivelakancane avela ezicutshini nasezimvilophini zemisipha (aponeuroses). Ukuthuthukiswa okungalindelekile, kungaba umthombo wobuhlungu kanye nokungakhululeki okuphawulekayo kokusebenza. Abaphathi bayinkimbinkimbi futhi badinga ukungenelela kwethimba lochwepheshe bemikhakha eminingi.

Incazelo

I-Desmoid tumors noma i-aggressive fibromatosis yizimila ezingavamile ezakhiwe ngamaseli ane-fibrous afana namangqamuzana avamile kumathishu ama-fibroblasts. Okuyingxenye yesigaba sezimila ezixhumeneyo (izimila “ezithambile” zezicubu), zikhula zisuka emisipha noma ezimvilophini zemisipha (aponeuroses).

Lawa amathumba amahle - awawona imbangela yama-metastases - kodwa eguquguqukayo engalindelekile, evame ukufakazela ukuthi ihlasela kakhulu endaweni futhi iphindelela kakhulu ngisho noma ezinye zivela kancane noma kungenzeka zihlehle ngokuzenzakalelayo.

Angavela noma kuphi emzimbeni. Amafomu angaphandle akhetha ukufinyelela ezithweni kanye nodonga lwesisu, kodwa intamo nekhanda (ezinganeni ezincane) noma i-thorax nakho kungaba isihlalo. Kukhona futhi izinhlobo ezijulile zezimila ze-desmoid (i-intra-abdominal localization).

Izimbangela

Imvelaphi yamathumba e-desmoid isalokhu iqondwa kabi, kodwa kucatshangwa ukuthi inezici eziningi, ngokubandakanyeka kwamahomoni kanye nezici zofuzo.

Ukuhlukumezeka ngengozi noma ukuhlinzwa (izibazi) kubonakala kungenye yezimbangela zokubukeka kwabo, kanye nokubeletha (ezingeni lodonga lwesisu).

kwesifo

Ukuhlolwa kwezithombe kubonisa ukuba khona kwenqwaba engena ngaphakathi ekhula ngokuhamba kwesikhathi. Ukuxilongwa kusekelwe kakhulu ku-CT (i-computed tomography noma i-CT) yamathumba angaphakathi kwesisu noma i-MRI (I-Magnetic Resonance Imaging) yezinye izimila.

I-biopsy iyadingeka ukuze kuqinisekiswe ukuxilongwa. Ukukhipha ingozi yokudideka, ukuhlaziywa kwe-histological (ukuhlolwa ngaphansi kwe-microscope) kufanele kwenziwe udokotela onguchwepheshe be-pathology onolwazi kulawa mathumba.

Ukuhlolwa kofuzo kungenziwa ngaphezu kokuthola uguquko olungase lube khona.

Abantu abathintekayo

Amathumba e-Desmoid ahlasela kakhulu abantu abasha, afinyelela inani eliphakeme cishe eminyakeni engama-30. Lesi sifo sihlasela abesifazane kakhulu. Izingane nazo kufanele zithinteke, ikakhulukazi lapho zisanda kweva eshumini nambili. 

Kuyithumba elingajwayelekile (0,03% lazo zonke izimila), elivela nemvamisa elinganiselwa minyaka yonke ezimweni ezintsha ezi-2 kuya kwezi-4 kuphela kwisigidi ngasinye.

Izici zengozi

Emindenini ehlaselwe i-adenomatous polyposis yomndeni, isifo esingavamile esitholakala ngofuzo esibonakala ngokuba khona kokuphindaphindeka kwekholoni, ingozi yokuthuthukisa isimila se-desmoid ingaphezu kwenani labantu elivamile futhi kulinganiselwa ukuthi cishe i-10 kuya ku-15%. Ihlotshaniswa nokuguqulwa kwezakhi zofuzo okuthiwa i-APC (i-tumor suppressor gene), ehilelekile kulesi sifo.

Kodwa-ke, iningi lezimo ze-aggressive fibromatosis zivela ngezikhathi ezithile (ngaphandle kwesizinda sofuzo). Cishe ku-85% walezi zimo ezingadluliseki, ukuguqulwa kwesimila samangqamuzana bese kuhlotshaniswa nokuguqulwa ngephutha kofuzo. I-CTNNB1, okubangela ukuguqulwa kwephrotheni ehilelekile ekulawuleni ukwanda kwesimila esibizwa ngokuthi i-beta-catenin.

Ukuvuvuka

Amathumba e-Desmoid adala ukuvuvukala okutholwa epalpation njengaqinile, ahambayo, ngezinye izikhathi "amabhola" amakhulu kakhulu avame ukunamathela ezakhiweni eziphilayo eziseduze.

ubuhlungu

Isimila asibuhlungu ngokwaso kodwa singabangela ubuhlungu obukhulu bemisipha, besisu noma bemizwa kuye ngokuthi ukuphi.

Izakhi zofuzo ezisebenzayo

Ukucindezelwa okwenziwa ezicutshini ezingomakhelwane kungabangela ukuphazamiseka okuhlukahlukene kokusebenza. Ukucindezelwa kwezinzwa kungaba, isibonelo, kube yimbangela yokuncipha kokuhamba kwesitho. Amafomu ajulile athinta imithambo yegazi, amathumbu noma uhlelo lomchamo, njll.

Ukulahlekelwa ukusebenza kwesitho esithintekayo kungenzeka.

Amanye ama-desmoid tumors nawo anomkhuhlane.

Alikho isu lokwelapha elisezingeni futhi kunqunywa icala ngalinye yithimba elinochwepheshe bemikhakha eminingi.

Izimila ze-desmoid ezinzile zingaba buhlungu futhi zidinga ukwelashwa kobuhlungu. 

Ukuqapha okusebenzayo

Okwakwenziwe ngaphambili, ukuhlinzwa manje sekuyekwa kuvumela indlela yokonga ehlanganisa ukuqapha ngokucophelela ukuvela kwesimila ngaphambi kokubeka ukwelashwa okunzima kwesinye isikhathi obekungeke kudingeke.

Ngisho nalapho isimila sizinzile, ukuphathwa kobuhlungu kungase kudingeke.

ukuhlinzwa

Ukususwa ngokuphelele kokuhlinzwa kwesimila se-desmoid kukhethwa uma kungenzeka futhi ukunwetshwa kwesimila kukuvumela ngaphandle kokubangela ukulahlekelwa okukhulu kokusebenza (isb. ukunqunywa isitho).

Radiotherapy

Ingase isetshenziselwe ukuzama ukwenza isimila se-desmoid sihlehle noma sisinzise, ​​uma kwenzeka siqhubeka, siphindelela noma ukunciphisa ingozi yokuphinda iphinde ngemva kokuhlinzwa. Ngenxa yemiphumela yayo eyingozi kubantu abakhulayo futhi isetshenziswa kancane kakhulu ezinganeni. 

Izidakamizwa zokwelapha

Ama-molecule ahlukene asebenza kahle kakhulu noma kancane futhi asetshenziswa ewodwa noma ehlangene. Ikakhulukazi, i-tamoxifen, isidakamizwa esilwa ne-estrogen esisebenzayo sisetshenziswa lapho isimila sizwela le hormone yesifazane, emithini ye-non-steroidal eqeda ukuvuvukala, ezinhlotsheni ezahlukene ze-chemotherapy (methotrexate, vinblastine / vinorelbine, pegylated liposomal doxorubicin) noma izidakamizwa eziqondiswe kumamolekyuli ezivimba ukukhula kwesimila (imatinib, sorafenib), ezinikezwe njengamaphilisi.

Ezinye izindlela zokwelapha

• I-Cryotherapy isetshenziswa ngaphansi kwe-anesthesia ejwayelekile ukuze ibhubhise izimila ngokuziqandisa

  -80 ° C.

• Ukujova esithweni okusodwa kuhlanganisa ukuthela umthamo omkhulu wokwelapha ngamakhemikhali esithweni esithintekile kuphela.

Lezi zinqubo zinikezwa kuphela ezikhungweni zochwepheshe ezimbalwa e-France.

Evolution

Ezimweni ezingaba ngu-70%, ukuphindaphinda kwendawo ye-tumor kuyabonwa. I-prognosis ebalulekile ayihlanganyeli, ngaphandle uma kwenzeka kuba nezinkinga zokuhlinzwa, ikakhulukazi izimila zesisu.