Zowopsa koma zobwerezabwereza komanso zomwe zimatha kukhala zowononga kwambiri, zotupa za desmoid, kapena aggressive fibromatosis, ndi zotupa zomwe zimayamba kuchokera ku minofu ndi ma envulopu a minofu (aponeuroses). Kukula kosayembekezereka, kumatha kukhala gwero la zowawa komanso kusapeza bwino kwantchito. Kuwongolera ndizovuta ndipo kumafuna kulowererapo kwa gulu la akatswiri odziwa zambiri.

Tanthauzo

Zotupa za Desmoid kapena aggressive fibromatosis ndi zotupa zosawerengeka zomwe zimapangidwa ndi ma cell a fibrous omwe amafanana ndi maselo abwinobwino mu minofu ya fibroblasts. Zamgulu la zotupa zolumikizira (zotupa "zofewa") zimayamba kuchokera ku minofu kapena ma envulopu a minofu (aponeuroses).

Izi ndi zotupa zowopsa - sizomwe zimayambitsa ma metastases - koma za chisinthiko chosadziwika bwino, chomwe nthawi zambiri chimakhala chosokoneza m'deralo komanso chobwerezabwereza ngakhale china chimasintha pang'ono kapena chikhoza kubwereranso mwadzidzidzi.

Amatha kuwuka paliponse m'thupi. Mawonekedwe apamwamba amafikira miyendo ndi khoma lamimba, koma khosi ndi mutu (mwa ana aang'ono) kapena thorax ingakhalenso mpando. Palinso mitundu yakuya ya zotupa za desmoid (intra-abdominal localization).

Zimayambitsa

Magwero a zotupa za desmoid sizikudziwika bwino, koma zimaganiziridwa kuti ndizochita zambiri, ndikuphatikizidwa kwa mahomoni ndi ma genetic factor.

Kuvulala mwangozi kapena opaleshoni (zipsera) zikuwoneka ngati chimodzi mwa zifukwa za maonekedwe awo, komanso kubereka (pa mlingo wa khoma la m'mimba).

matenda

Kuyesa kwazithunzi kukuwonetsa kukhalapo kwa misa yolowera yomwe imakula pakapita nthawi. Matendawa makamaka amachokera ku CT (computed tomography kapena CT) ya zotupa za m'mimba kapena MRI (Magnetic Resonance Imaging) ya zotupa zina.

Biopsy imafunika kuti mutsimikizire za matendawa. Kuti apewe kusokonezeka, kusanthula kwa histological (kuwunika pa microscope) kuyenera kuchitidwa ndi dokotala wodziwa za matenda odziwa zotupa izi.

Kuyeza kwa majini kumatha kuchitidwa kuwonjezera pakuwona kusintha komwe kungatheke.

Anthu okhudzidwa

Zotupa za Desmoid zimakhudza kwambiri achinyamata, zomwe zimafika pachimake zaka 30. Matendawa amakhudza kwambiri amayi. Ana nawonso amakhudzidwa, makamaka achichepere. 

Ndi chotupa chosowa (0,03% ya zotupa zonse), kuwoneka pafupipafupi pafupifupi chaka chilichonse pamilandu 2 mpaka 4 yatsopano pa anthu miliyoni miliyoni.

Zowopsa

M'mabanja omwe akhudzidwa ndi adenomatous polyposis, matenda osowa cholowa omwe amadziwika ndi kukhalapo kwa machulukidwe a m'matumbo, chiopsezo chokhala ndi chotupa cha desmoid chimakhala chachikulu kuposa anthu ambiri ndipo akuti pafupifupi 10 mpaka 15%. Zimagwirizanitsidwa ndi kusintha kwa jini yotchedwa APC (tumor suppressor gene), yomwe imakhudzidwa ndi matendawa.

Komabe, milandu yambiri ya aggressive fibromatosis imawoneka mwa apo ndi apo (popanda cholowa). Pafupifupi 85% ya milandu yosapatsirana iyi, kusintha kwa chotupa kwa maselo kumalumikizidwa ndi kusintha kwangozi kwa jini. Chithunzi cha CTNNB1, kupangitsa kusinthidwa kwa puloteni yomwe imakhudzidwa ndi kuwongolera kuchuluka kwa chotupa chotchedwa beta-catenin.

kutupa

Zotupa za Desmoid zimapanga kutupa komwe kumadziwika pa palpation ngati "mipira" yolimba, yoyenda, nthawi zina yayikulu kwambiri yomwe nthawi zambiri imamatira kuzinthu zapafupi.

ululu

Chotupacho sichimapweteka pachokha koma chingayambitse kupweteka kwakukulu kwa minofu, m'mimba kapena mitsempha malinga ndi malo ake.

Majini ogwira ntchito

Kuponderezedwa komwe kumachitika pa minofu yoyandikana nayo kungayambitse zovuta zosiyanasiyana. Kuponderezana kwa mitsempha kumatha, mwachitsanzo, kukhala chifukwa cha kuchepetsa kuyenda kwa mwendo. Mitundu yakuya imakhudza mitsempha ya magazi, matumbo kapena mkodzo, ndi zina zotero.

Kutayika kwa ntchito ya chiwalo chokhudzidwa ndi kotheka.

Zotupa zina za desmoid zimakhalanso ndi malungo.

Palibe njira zochiritsira zokhazikika ndipo zimaganiziridwa pazochitika ndi gulu la akatswiri odziwa zambiri.

Zotupa zokhazikika za desmoid zimatha kukhala zowawa ndipo zimafuna chithandizo chamankhwala. 

Kuyang'anira mwachangu

Opaleshoni yomwe idachitidwapo kale, tsopano yasiyidwa m'malo mwa njira yodziyimira pawokha yomwe imaphatikizapo kuyang'anira kusinthika kwa chotupacho musanapereke chithandizo cholemera chomwe sichingakhale chofunikira.

Ngakhale chotupacho chitakhala chokhazikika, kuchepetsa ululu kungakhale kofunikira.

opaleshoni

Kuchotsa kotheratu kwa chotupa cha desmoid kumakondedwa ngati n'kotheka ndipo kutambasula kwa chotupacho kumalola popanda kuwononga kwambiri ntchito (mwachitsanzo, kudulidwa kwa mwendo).

Radiotherapy

Itha kugwiritsidwa ntchito kuyesa kupangitsa chotupa cha desmoid kuti chibwererenso kapena kuchikhazikitsira, pakapita patsogolo, kuyambiranso kapena kuchepetsa chiopsezo choyambiranso pambuyo pa opaleshoni. Chifukwa cha zotsatira zake zoipa pa kukula anthu ndi ntchito zochepa kwambiri ana. 

Mankhwala osokoneza bongo

Mamolekyu osiyanasiyana amakhala ndi mphamvu zochulukirapo kapena zochepa ndipo amagwiritsidwa ntchito pawokha kapena kuphatikiza. Makamaka, tamoxifen, mankhwala oletsa anti-estrogen amagwiritsidwa ntchito pamene chotupacho chimakhudzidwa ndi hormone yachikazi iyi, mankhwala osagwiritsidwa ntchito ndi anti-inflammatory, ku mitundu yosiyanasiyana ya chemotherapy (methotrexate, vinblastine / vinorelbine, pegylated liposomal doxorubicin) kapena mankhwala ochizira mamolekyulu omwe amaletsa kukula kwa chotupa (imatinib, sorafenib), operekedwa ngati mapiritsi.

Mankhwala ena

• Cryotherapy imagwiritsidwa ntchito pansi pa anesthesia wamba kuti awononge zotupa poziziritsa mkati

  -80 ° C.

• Kulowetsedwa kwa miyendo kwapayokha kumaphatikizapo kulowetsa mankhwala amphamvu kwambiri m'mbali yomwe yakhudzidwa yokha.

Njirazi zimangoperekedwa m'malo ochepa akatswiri ku France.

Evolution

Pafupifupi 70% ya milandu, kubwereza kwa chotupa kumawonedwa. Chofunika kwambiri cha matendawa sichimagwiritsidwa ntchito, kupatula ngati pali zovuta za opaleshoni, makamaka zotupa za m'mimba.