I-Dermatofibrosarcoma protuberans (DFSP)

ngu-Glenda Wright MBBCh kanye no-Allison Osmond MD FRCPC
Mashi 9, 2023

Iyini i-dermatofibrosarcoma protuberans?

I-Dermatofibrosarcoma protuberans (DFSP) wuhlobo lomdlavuza wesikhumba oqala ngaphansi nje kobuso isikhumba. I-DFSP ingaphinde ikhule endaweni efanayo ngemva kokuhlinzwa ikakhulukazi uma isimila singazange sikhishwe ngokuphelele phakathi nokuhlinzwa kokuqala. Nokho, ngokungafani nezinye izinhlobo zomdlavuza, i-DFSP ayivamile neze i-metastases (isakazeka) kwezinye izingxenye zomzimba.

Hlobo luni lomdlavuza i-dermatofibrosarcoma protuberans?

I-DFSP iwuhlobo lwe i-sarcoma.

I-dermatofibrosarcoma protuberans ivame ukutholakala kuphi?

Izimila eziningi ziqala esifubeni noma emhlane kodwa noma iyiphi ingxenye yomzimba ingathinteka.

Yiziphi izimpawu ze-dermatofibrosarcoma protuberans?

Ezigulini eziningi, isimila sikhula kancane futhi asibuhlungu.

Yini ebangela i-dermatofibrosarcoma?

I-DFSP ibangelwa ukuguquka kofuzo okuholela ekuhlanganisweni (inhlanganisela) yezakhi zofuzo ezimbili i-COL1A1 ne-PDGFB. Lolu shintsho lwezakhi zofuzo luphumela ekukhuleni kokukhiqizwa kwephrotheni ye-PDGFB nokuthuthukiswa kwesimila. Akwaziwa ukuthi kungani abanye abantu beba nalolu shintsho lofuzo.

Ingabe i-dermatofibrosarcoma protuberans isakazeka kwezinye izingxenye zomzimba?

Ama-DFSP ajwayelekile cishe awasoze i-metastasis (sabalala) kwezinye izingxenye zomzimba. Kodwa-ke, i-fibrosarcomatous DFSP iwuhlobo lomdlavuza olunolaka olungakwazi ukumetastases.

Itholakala kanjani i-dermatofibrosarcoma protuberans?

Ukuxilongwa kuvame ukwenziwa ngemva kokukhishwa kwesampula yethishu encane ngenqubo ebizwa ngokuthi a i-biopsy. Ukuxilongwa kungenziwa futhi ngemva kokukhishwa kwesimila sonke ngenqubo ebizwa ngokuthi i ukuziqhenya. Uma ukuxilongwa kwenziwa ngemva kwe-biopsy, udokotela wakho cishe uzoncoma inqubo yokuhlinzwa yesibili ukuze asuse lonke isimila.

I-dermatofibrosarcoma protuberans ibukeka kanjani ngaphansi kwesibonakhulu?

Ngaphansi kwe-microscope, i-DFSP yenziwe ngamaseli amade azacile abizwa amaseli okuphotha ezibukeka zifana kakhulu nama-fibroblasts avame ukutholakala ku-dermis of the isikhumba. Amaseli okuphotha akhula ngendlela eyindilinga izazi zezifo eziyibiza ngokuthi “spoke and wheel” noma “cartwheel” iphethini. Isimila siqala ku-dermis, kodwa amangqamuzana omdlavuza ngokuvamile atholakala ekhula abe amafutha angaphansi kwesikhumba ngezansi. I-pathologist yakho ingenza ukuhlolwa okubizwa i-immunohistochemistry ukusiza ukuqinisekisa ukuxilongwa. Amaseli wesimila ku-DFSP ngokuvamile alungile I-CD34 kanye negethivu ku-Factor 13A.

Kusho ukuthini uma i-dermatofibrosarcoma protuberans ichazwa njenge-fibrosarcomatous?

Izazi zezifo zisebenzisa igama elithi fibrosarcomatous ukuchaza i-DFSP esishintshe yaba isimila esinolaka. Ngokungafani ne-DFSP ejwayelekile, i-fibrosarcomatous DFSP inamandla okwenza i-metastasis (sabalala) kwezinye izingxenye zomzimba. Uma ihlolwa ngaphansi kwesibonakhulu, i-fibrosarcomatous DFSP ingase ibonise iphethini ye-fascicular noma i-herringbone yokukhula. Amaseli wesimila angase avele kakhulu okuhlekisayo (ukubukeka ngendlela engavamile ngesimo noma usayizi) kanye izibalo ze-mitotic (amaseli wesimila ahlukana ukuze akhe amaseli amasha wesimila) avame ukubonakala

Iyini imajini?

A wekhasi yinoma yisiphi isicubu esisikwe udokotela ohlinzayo ukuze akhiphe isimila emzimbeni wakho. Noma nini lapho kungenzeka, odokotela abahlinzayo bazozama ukusika izicubu ngaphandle kwesimila ukuze banciphise ingozi yokuthi noma yimaphi amangqamuzana esimila azosala ngemva kokukhishwa kwesimila. Udokotela wakho wezifo uzohlola ngokucophelela wonke amaphethelo kusampula yakho yethishu ukuze abone ukuthi amaseli wesimila asondele kangakanani emaphethelweni ezicubu ezisikiwe. Amamajini azochazwa kuphela embikweni wakho ngemva kokuba isimila sesisusiwe.

I-DFSP inamandla okuphinda ikhule (iphindeke), ikakhulukazi uma ingasuswanga ngokuphelele. Umngcele ophakathi kwesimila kanye nezicubu ezizungezile ezivamile kungaba nzima ukuwubona ngenxa yokuthi amaseli wesimila ku-DFSP avame ukunwebeka njengamaqembu amancane amaseli emaphethelweni eqhubu aye esicutshini esivamile esizungezile. Lokhu kungenza ukususa isimila kube inselele njengoba udokotela wakho ohlinzayo engase angazi ukuthi isimila siphelelaphi.

Imajini eyinegethivu isho ukuthi awekho amaseli wesimila emaphethelweni ezicubu ezisikiwe. Uma wonke amamajini engemihle, imibiko eminingi ye-pathology izosho ukuthi amaseli wesimila asondele kakhulu abekude kangakanani kumajini. Ibanga ngokuvamile lichazwa ngamamilimitha. Imajini ibhekwa njengenhle lapho kunamaseli anesimila emaphethelweni ezicubu ezisikiwe. Umkhawulo omuhle uhlotshaniswa nengozi enkulu yokuthi isimila siphinde sivele endaweni efanayo ngemva kokwelashwa.