He aha te DSRCT? Ngā tohu, ngā take, me te rongoā

Ko te DSRCT te kupu poto mō te Desmoplastic Small Round Cell Tumor, he momo mate pukupuku onge ngākaha e pā ana ki ngā tāngata taitamariki. Ko tēnei mate pukupuku onge e tupu ana i te puku, ina koa i te peritoneum (te paparinga o te kohanga o te puku), ahakoa ka puta pea i ētahi atu wāhanga o te tinana.

Ahakoa he onge te DSRCT, e pā ana ki ngā i raro iho i te 200 o te tangata puta noa i te ao ia tau, ko te mārama ki tēnei āhuatanga ka taea te āwhina i a koe ki te mohio ki ngā tohu pea, me te mohio ki te wā hei kimi atu i te awhina hauora. Ko te nuinga o ngā take e puta ana i ngā taitamariki me ngā tāngata kōriki, me ngā tane e whā tekau te maha atu i ngā wāhine.

He aha te DSRCT?

Ko te DSRCT he sarcoma kiko ngohengohe e noho ana ki roto i tētahi rōpū o ngā mate pukupuku e kiia nei ko ngā mate pukupuku pōro iti. Ka whiwhi te mate pukupuku i tōna ingoa mai i ngā āhuatanga matua e rua: kei roto he pōro iti, ngā pūtau mate pukupuku, ā, kei te taha o te kiko ngohengohe kaha e kiia nei ko te desmoplastic stroma.

Ko tēnei mate pukupuku e tupu ana hei maha o ngā kohinga puta noa i te kohanga o te puku, kāore i te mea kotahi te mate pukupuku. Ka rereke te rahi o ngā kohinga, ā, ka horapa pinepine i runga i ngā mata o te peritoneum, koia te take i karangatia ai i etahi wā he "peritoneal sarcomatosis."

Ko te mea e whakaatu ana i te DSRCT he ahua motuhake tōna hanganga ira. Kei roto i ngā pūtau mate pukupuku he translocation chromosomal āhuatanga e hanga ana i tētahi pūtau whakakotahi koretake, e arahi ana i te tipu o te mate pukupuku me te whāinga kaha.

He aha ngā tohu o te DSRCT?

Ka taea e ngā tohu o te DSRCT te tino ngāwari, ā, ka tupu pea i roto i ngā wiki, i ngā marama rānei. Ko te nuinga o te iwi e whakakahore ana i ēnei tohu hei take iti o te pūnaha kai, i ngā raruraru rānei e pā ana ki te ahotea.

Ko ngā tohu tino noa ka pā ki a koe:

• Te mamae o te puku, te kore rongoa rānei e kore e ngaro me te okioki
• Te pupuhi o te puku, te pupuhi rānei e kino haere ana i te wā
• Te ahua o te makona ahakoa te kai iti
• Te ngaro o te taumaha kaore he take ahakoa te hiahia kai noa, te nui rānei
• Te ruaki, te ruaki rānei, ina koa i muri i ngā kai
• Ngā huringa i ngā tikanga o te whekau, tae atu ki te kōpuku, te uaua rānei ki te tukunga o te paru
• Te ngenge e kore e pai ake me te okioki
• Te ngaro o te hiahia kai

I roto i ngā take whanui ake, ka kite pea koe i te kohinga e taea te kite i roto i tō puku e taea te kite i roto i tō kiri. Ko ētahi atu tangata e pā ana ki te poto o te manawa ki te kohikohi o te wai i roto i te kohanga o te puku, he āhuatanga e kiia nei ko te ascites.

He mea nui kia mahara ko ēnei tohu ka puta mai i ngā āhuatanga maha, ko te nuinga he tino noa, he iti rawa te kino atu i te DSRCT. Heoi, ki te pā ana ki a koe ētahi o ēnei tohu, he mea tika kia kōrero ki tō kaiwhakarato hauora.

He aha ngā take o te DSRCT?

Ko te take tika o te DSRCT kāore i te tino mohiotia, ka taea te whakaaro pōuri ki te whakamārama i te take i puta ai tēnei mate pukupuku. Ko te mea e mohiotia ana e tatou ko te DSRCT e puta mai ana i te huringa ira motuhake e puta ana i ngā pūtau.

Ko tēnei huringa ira e pā ana ki te translocation i waenganui i ngā chromosomes 11 me te 22, e hanga ana i te gene whakakotahi koretake e kiia nei ko te EWSR1-WT1. Ko tēnei gene whakakotahi e whakaputa ana i te pūtau e whakaroa ana i te tipu o te pūtau me te wehenga, e arahi ana ki te whanaketanga o ngā pūtau mate pukupuku.

Kāore i rite ki ētahi atu mate pukupuku, kāore te DSRCT e hono atu ki:

• Ngā āhuatanga ira e tuku ana, i te hītori whānau rānei
• Ngā whakaū taiao, i ngā āhuatanga noho rānei
• Ngā maimoatanga radiation, i te chemotherapy rānei i mua
• Ngā mate huaketo
• Ngā tikanga kai, i te whakakori tinana rānei

Ko te huringa ira e puta ai te DSRCT he mahi whakahirahira e puta ana i te wā e wehewehe ana te pūtau. Ko te tikanga o te whanaketanga o te DSRCT ehara i te mea ka taea te aukati ma ngā whiringa rerekē, i ngā whāinga rānei.

Nōhea te kite i te taote mō ngā tohu DSRCT?

Me whakaaro koe ki te kite i tō taote ki te pā ana ki a koe ngā tohu puku e mau tonu ana i te rua wiki neke atu, ina koa ki te kino haere ana. Ahakoa he tino noa ake ēnei tohu e puta mai ana i ngā āhuatanga noa, he pai tonu te arotake i a rātou.

Rapua te awhina hauora kia tere ake ki te pā ana ki a koe:

• Te mamae o te puku, te mamae rānei
• Te pupuhi o te puku i roto i ngā rā, kāore i ngā wiki
• Te ruaki e ārai ana i a koe ki te pupuri i te kai, i ngā wai rānei
• Ngā tohu o te aukatinga o te whekau, pēnei i te kore e taea te tukunga o te hau, te nekehanga rānei o te whekau
• Te ngaro o te taumaha kaore he take (neke atu i te 10 pauna kaore he whakamātautau)
• Te uaua ki te manawa me te pupuhi o te puku

Kia mahara kei reira tō kaiwhakarato hauora hei āwhina i a koe ki te wehewehe i ngā tohu e awangawanga ana. Ka taea e rātou te mahi i ngā whakamātautau tika hei whakatau i te take o ō tohu, ā, ka hoatu ki a koe te mahere maimoatanga tika.

He aha ngā take morearea mō te DSRCT?

Ko te DSRCT he iti rawa ngā take morearea e taea te whakatau, he mea whakamarie, he mea miharo hoki ki ngā kairangahau hauora. E puta ana te mate pukupuku i te mutunga o te mahi whakahirahira, kāore i te hono atu ki ngā take morearea e taea te whakahaere.

Ko ngā take morearea matua kua kitea ko:

• Te tau: Ko te nuinga o ngā take e puta ana i ngā tāngata i waenga i te 10 me te 30 tau te pakeke, me te pikinga o te mate i ngā tau tekau whakamutunga me ngā tekau tau tuatahi
• Te ira tangata: E whā te maha atu o ngā tane i ngā wāhine
• Kāore he tauira whakatūpato e mohiotia ana: Kāore te DSRCT e rere i roto i ngā whānau

Kāore i rite ki te maha atu o ngā mate pukupuku, kāore te DSRCT e hono atu ki te whakakī, te inu waipiro, te kai, te whakakori tinana, ngā whakaū mahi, i ngā maimoatanga hauora o mua rānei. Ka taea tēnei te whakamarie, no te mea he iti te mea ka taea e koe te mahi rerekē hei aukati i a ia.

Ko te onge o tēnei mate pukupuku te tikanga hoki, ahakoa ngā tāngata i roto i ngā rōpū morearea teitei (ngā tāne taitamariki) he iti rawa te tūpono ki te whanake i te DSRCT. Ko te morearea whānui e iti ana i te 1 i roto i te miriona o te tangata ia tau.

He aha ngā raruraru pea o te DSRCT?

Ka taea e te DSRCT te arahi ki ngā raruraru maha, na te tupu me te horapa atu ki te kohanga o te puku. Ko te mārama ki ēnei raruraru pea ka taea te āwhina i a koe ki te mohio ki te wā ka tino kino haere ngā tohu.

Ko ngā raruraru tino noa ko:

• Te aukatinga o te whekau: Ka taea e ngā mate pukupuku te aukati i ngā whekau, e ārai ana i te kai noa
• Ascites: Te kohikohi wai i roto i te kohanga o te puku e puta ai te pupuhi me te kore rongoa
• Te kore kai: Na te kore hiahia kai, te ruaki, me te uaua ki te kai
• Ngā toto: Ka taea e te mate pukupuku te whakapiki i te morearea o ngā kohinga e hanga ana i roto i ngā oko toto
• Ngā raruraru whatukuhu: Ki te pehi, ki te whakauru rānei ngā mate pukupuku ki te pūnaha mimi

I roto i ngā take whanui ake, ka taea e te DSRCT te horapa atu i te kohanga o te puku ki ētahi atu whekau, ko te nuinga ko te ate, ngā pūkahukahu, i ngā ngota lymph rānei. Heoi, ko tēnei momo horapa tawhiti he iti ake te noa i te horapa rohe i roto i te puku.

He mea nui kia mohio ko te tiaki tautoko hou ka taea te whakahaere pai i ēnei raruraru, hei āwhina i te pupuri i te kounga o te ora i te wā e maimoatia ana. Ka tirotirohia e tō rōpū hauora ēnei take, ā, ka whakaotihia e rātou kia tere ake ki te puta.

Me pēhea te whakatau i te DSRCT?

Ko te whakatau i te DSRCT e tika ana kia maha ngā mahi, no te mea me whakakahore e ngā taote ngā āhuatanga noa i mua. Ko te tukanga e tīmata ana i tō hītori hauora me te whakamātautau tinana o tō puku.

Ka tono pea tō taote i ngā whakamātautau whakaahua hei tiki i tētahi tirohanga pai ake o te mea e puta ana i roto i tō puku. Ko te CT scan o te puku me te pelvis te whakamātautau whakaahua tuatahi e mahia ana, no te mea ka taea te whakaatu i te rahi, te wāhi, me te maha o ngā kohinga kei reira.

Ka uru pea ngā whakamātautau atu ki:

• Ngā whakaahua MRI mō ngā whakaahua taipitopito o ngā kiko ngohengohe
• Ngā whakaahua PET hei tirotiro i te mahi mate pukupuku puta noa i te tinana
• Ngā whakamātautau toto hei tirotiro i tō hauora whānui me te mahi o ngā whekau
• Ngā tohu mate pukupuku, ahakoa kāore ēnei e motuhake ki te DSRCT

Ko te whakatau whakamutunga e hiahia ana ki te biopsy, kei hea te tauira iti o te kiko ka tangohia ka tirotirohia i raro i te microscope. Ka titiro te pathologist ki ngā pūtau pōro iti āhuatanga, ā, ka mahi i ngā whakamātautau motuhake hei whakau i te gene whakakotahi EWSR1-WT1 e whakatau ana i te DSRCT.

Ka taea e tēnei tukanga whakatau te torutoru rā ki ngā wiki, ka taea te whakaaro pōuri. Kia mahara ko tēnei huarahi tino pai e whakarite ana ka whiwhi koe i te whakatau tino tika, he mea nui mō te whakarite i te rautaki maimoatanga pai.

He aha te maimoatanga mō te DSRCT?

Ko te maimoatanga mō te DSRCT e tika ana kia maha ngā mahi e whakakotahi ana i ngā momo rongoā rerekē. Ko te whāinga ko te whakaiti i ngā mate pukupuku kia taea ai, ā, kia whakahaerehia te mate mo te wā roa.

Ko te huarahi maimoatanga paerewa e whakauru ana ki:

1. Chemotherapy: Ngā rauna maha o ngā rongoā mate pukupuku kaha e hoatu ana i roto i ngā marama maha
2. Te pokanga: Te tango i te nui o te mate pukupuku kia taea ai, e whakakotahitia ana me te chemotherapy wera tika i roto i te puku
3. Te rongoā radiation: Ngā hihi kaha teitei e whakapae ana ki ngā pūtau mate pukupuku e toe ana

Ko te wāhanga chemotherapy e tīmata ana i te tuatahi, ā, ka roa pea te 4-6 marama. Ko ngā whakakotahitanga rongoā noa ko te ifosfamide, te carboplatin, te etoposide, me te doxorubicin. Ko ēnei rongoā e mahi ana ma te whakapae i ngā pūtau mate pukupuku e wehewehe ana.

Ko te pokanga, ki te taea, e whakauru ana ki te tukanga e kiia nei ko te pokanga cytoreductive me te hyperthermic intraperitoneal chemotherapy (HIPEC). Ko tēnei e whakauru ana ki te tango i ngā mate pukupuku e kitea ana, katahi ka horoi i te kohanga o te puku me ngā rongoā chemotherapy wera.

I te wā e maimoatia ana, ka arotahi hoki tō rōpū hauora ki te tiaki tautoko hei āwhina i te whakahaere i ngā pānga, me te pupuri i tō kaha me te kounga o te ora. Ka uru pea tēnei ki ngā rongoā mō te ruaki, te tautoko kai, me ngā maimoatanga hei ārai i ngā mate.

Me pēhea te whakahaere i ngā tohu i te kāinga i te wā e maimoatia ana te DSRCT?

Ko te whakahaere i ngā tohu i te kāinga ka taea te āwhina i a koe ki te pai ake te ahua, me te pupuri i tō kaha i te wā e maimoatia ana. Ko ngā whakatikatika iti o ia rā ka taea te whakarereke nui i tō ahua whānui.

Mō ngā tohu pūnaha kai, ko te kai iti, te maha atu o ngā kai e mahi pai ana i te whakamātautau ki te kai nui. Aroha ki ngā kai ngāwari ki te kai, me te pai ki a koe, ahakoa kāore ō kai tino pai e pai ana inaianei.

Hei whakahaere i te ngenge:

• Okioki ki te hiahia koe, engari kia ngāwari te mahi i te wā e taea ai
• Haere i ngā hikoi poto, i te whakakorikori māori rānei kia taea ai
• Pupuri i te wa okioki noa i te wā e taea ai
• Pātai atu ki ngā whānau me ngā hoa mō te āwhina ki ngā mahi o ia rā

Mō te ruaki me ngā take hiahia kai, whakamātautau ki te kai i ngā kai ngāwari pēnei i ngā keke, te toki, i te raihi rānei. Ko te tii karika, i ngā taapiri karika rānei ka āwhina pea ki te ruaki. Kia makona te inu wai ma te inu iti o ngā wai puta noa i te rā.

Tāruahia ō tohu me ō pānga kia taea ai te kōrero ki tō rōpū hauora. Ka taea e rātou te whakarereke i ngā rongoā, i ngā maimoatanga tautoko atu rānei hei āwhina i a koe ki te pai ake te ahua.

Me pēhea te whakarite mō ō hui taote?

Ko te whakarite mō ō hui hauora ka taea te āwhina i a koe ki te whakamahi pai i tō wā me tō rōpū hauora, ā, kia whakarite ai ka whakautua ō pātai katoa. Ko te whakarite iti ka nui te āwhina i a koe ki te pai ake te whakahaere i tō tiaki.

I mua i ia hui, tuhia ō tohu o nāianei, tae atu ki te wā i tīmata ai rātou me te āhua o te huringa. Tātarihia ngā tohu hou, i ngā pānga rānei e pā ana ki a koe mai i te maimoatanga.

Whakaritea tētahi rārangi o ngā pātai e hiahia ana koe ki te ui:

• He aha ngā hua o oku whakamātautau hou?
• Me pēhea te mahi o taku maimoatanga?
• He aha ngā pānga ka titiro atu ahau?
• He huarahi kei reira hei whakahaere pai ake i aku tohu o nāianei?
• Nōhea taku maimoatanga e whai ake nei?

Kawe mai i te rārangi katoa o ngā rongoā katoa e inu ana koe, tae atu ki ngā rongoā-a-te-kāinga me ngā taapiri. Whakaarohia te kawe mai i tētahi mema whānau, i tētahi hoa rānei hei āwhina i a koe ki te mahara ki te mea i kōrerotia, me te whakarato i te tautoko ngākau.

Kaua e whakaroa ki te ui mō te whakamārama ki te kore koe e mārama ki tetahi mea. E hiahia ana tō rōpū hauora kia tino mārama koe ki tō āhuatanga me tō mahere maimoatanga.

He aha te mea nui mō te DSRCT?

Ko te DSRCT he mate pukupuku onge, engari he kino, e pā ana ki ngā tāngata taitamariki. Ahakoa ka taea te whakaaro pōuri ki te whakatau, kua pai ake ngā hua o te maimoatanga mō te maha o ngā turoro i ngā tau tata nei.

Ko te mea nui kia mahara kāore koe e noho kotahi i roto i tēnei haerenga. Kei a tō rōpū hauora te wheako ki te maimoatanga i tēnei mate pukupuku onge, ā, ka mahi tahi rātou me koe hei whakawhanake i te mahere maimoatanga pai rawa atu mō tō āhuatanga motuhake.

Ko te mohiotanga wawe o ngā tohu me te awhina hauora tere ka taea te whakarereke i ngā hua o te maimoatanga. Ki te pā ana ki a koe ngā tohu puku e mau tonu ana, ina koa he taitamariki koe, kaua e whakaroa ki te kōrero ki tō kaiwhakarato hauora.

Kia mahara ko te whai i ngā tohu e awangawanga ana kāore e tikanga kei a koe te DSRCT. He tino onge tēnei mate pukupuku, ā, he tino noa ake ngā tohu e puta mai ana i te āhuatanga noa, e taea te whakaora. Heoi, ko te arotake i a koe ka hoatu ki a koe te rangimarie o te ngākau, ā, ka whakarite kia whiwhi koe i te tiaki tika ahakoa te take.

Ngā pātai e ui pinepinetia ana mō te DSRCT

He whakatūpato te DSRCT?

Kāore, ehara te DSRCT i te whakatūpato, kāore hoki e rere i roto i ngā whānau. Ko ngā huringa ira e puta ai tēnei mate pukupuku e puta ana i te mutunga o te mahi whakahirahira i te wā e wehewehe ana te pūtau. Ko te whai i tētahi mema whānau me te DSRCT kāore e whakapiki i tō morearea ki te whanaketanga.

He onge rawa te DSRCT?

He tino onge te DSRCT, me ngā i raro iho i te 200 o ngā take hou e whakatauhia ana puta noa i te ao ia tau. Hei whakarite i tēnei, he tino nui ake te tūpono ki te patua e te uira i te whanaketanga o te DSRCT. Ko tēnei onge te take i uaua ai te whakatau i te tīmatanga.

Ka taea te whakaora i te DSRCT?

Ahakoa he mate pukupuku kaha te DSRCT, ko ētahi o ngā turoro e whiwhi ana i te whakahokinga wā roa me te maimoatanga kaha. Ko te whakakotahitanga o te chemotherapy, te pokanga, me te rongoā radiation kua āwhina i ētahi tangata ki te ora kore mate pukupuku mō ngā tau maha. Ko ngā hua o te maimoatanga e mau tonu ana te pai ake i te ako atu ngā taote ki tēnei mate pukupuku onge.

He aha te tau noa e whakatauhia ai te DSRCT?

Ko te nuinga o ngā tāngata me te DSRCT e whakatauhia ana i waenga i ngā tau 10 me te 30, me te maha rawa o ngā take e puta ana i ngā tau tekau whakamutunga me ngā tekau tau tuatahi. Heoi, kua pūrongohia ngā take i roto i ngā tamariki iti i te 5 me ngā pakeke tae atu ki te 50, ahakoa he iti rawa ēnei.

E hia te roa o te maimoatanga DSRCT?

Ko te tukanga maimoatanga katoa e roa ana i te 12-18 marama, ahakoa ka rereke tēnei i runga i ngā āhuatanga motuhake. E whakauru ana tēnei ki ngā marama maha o te chemotherapy, ka whai ake te pokanga (ki te taea), katahi ka whai ake te chemotherapy, i te rongoā radiation rānei. Ka hoatu e tō rōpū hauora tētahi wa wā roa atu i runga i tō mahere maimoatanga.