Aia kekahi wahi ʻē ma kou ʻili? Hiki paha iā ia ke lilo i DFSP (Dermatofibrosarcoma Protuberans)!

Ua ʻike anei ʻoe i kahi puʻupuʻu liʻiliʻi a i ʻole ke kala ʻana o ka ʻili ma kou ʻili? I kekahi manawa, ʻaʻole mākou e nānā nui iā ia, ʻeā? Akā i kekahi manawa, ʻoiai kekahi mea liʻiliʻi e like me kēia, hiki ke lilo i mea e hopohopo ai. I kēia lā, e kamaʻilio mākou e pili ana i kahi ʻano maʻi ʻili kakaʻikahi akā koʻikoʻi. Ua kapa ʻia ʻo Dermatofibrosarcoma Protuberans , a i ʻole DFSP no ka pōkole.

He aha ka DFSP (Dermatofibrosarcoma Protuberans)?

I ka ʻōlelo maʻalahi, he ʻano maʻi ʻili lohi ka ulu ʻana o ka DFSP, akā he ʻano kakaikahi iki. Hoʻomaka ia ma ka papa waena o ko kākou ʻili, ʻo ia hoʻi ka dermis . Hiki paha iā ʻoe ke lohe i ke kauka e kapa ana iā ia he DFSP.

No ka mea, he maʻi kanesa lohi kēia, ʻaʻole ia e laha pinepine i nā wahi ʻē aʻe o ke kino. Eia nō naʻe, ʻaʻole ia he manaʻo maikaʻi e hoʻowahāwahā iā ia. He mea nui loa ka loaʻa ʻana o ka lāʻau kūpono a wehe i kēia puʻupuʻu. A laila wale nō e hiki ai ke pale aku i ke kanesa mai ka hoʻi hou ʻana mai (recurrence) a i ʻole ka laha ʻana i nā wahi ʻē aʻe o ke kino (metastatic cancer). Me ka lāʻau kūpono, loaʻa i ke kanaka me DFSP kahi manawa kūpono loa e hoʻōla ai.

He puʻupuʻu wale nō anei kēia DFSP? A i ʻole he maʻi kanesa paha?

ʻAʻole, ʻaʻole wale ka DFSP he puʻupuʻu maikaʻi ʻole. He maʻi ʻino ia. ʻO ka pololei, he ʻano maʻi ʻaʻai palupalu ia, he ʻano sarcoma palupalu . ʻO nā Sarcomas nā maʻi ʻaʻai e hana ʻia ma nā wahi e like me ke ʻiʻo, ka momona, a me ka ʻili. I kekahi manawa, hiki iā lākou ke hoʻopilikia i nā iwi.

Hiki i ka DFSP ke laha (metastasize) i nā ʻāpana ʻē aʻe o ke kino?

ʻAʻole ʻo DFSP kahi ʻano maʻi kanesa e laha koke ana ma ke kino holoʻokoʻa. Eia nō naʻe, he mea kākaʻikahi loa, ma kahi o hoʻokahi i loko o 20 mau kānaka me DFSP hiki ke laha ka maʻi kanesa ma waho o kahi i hoʻomaka mua ai. Ua kapa ʻia kēia he kanesa metastatic .

ʻOi aku ka palahalaha o kēia inā ʻaʻole mālama ʻia, a inā paha ua pālahalaha hohonu ke kanesa i loko o ka ʻili, i loko o nā ʻiʻo momona a me nā ʻiʻo.

ʻO kekahi mea, ʻoiai ʻaʻole ʻike ʻia ke kumu pololei, inā ulu ka DFSP i ka wā hāpai, hiki ke lilo i mea ʻoi aku ka koʻikoʻi.

Eia kekahi, hiki i kahi poʻe liʻiliʻi me DFSP ke hoʻomohala i kahi ʻano ʻoi aku ka ikaika a wikiwiki hoʻi e laha ana i kapa ʻia ʻo fibrosarcomatous dermatofibrosarcoma protuberans (DFSP-FS) . ʻOiai inā e wehe ʻia kēia mau puʻupuʻu DFSP-FS ma ke ʻoki ʻana, ʻoi aku ka nui o ko lākou hoʻi ʻana mai a hiki ke laha i nā wahi ʻē aʻe.

Pehea ka maʻamau o kēia kūlana i kapa ʻia ʻo DFSP?

He ʻano maʻi kanesa laha ʻole ka DFSP. Ma ka honua holoʻokoʻa, hoʻopilikia wale ia i ʻehā mau kānaka no kēlā me kēia miliona mau kānaka i kēlā me kēia makahiki. ʻO DFSP ka helu ma waena o 1% a me 6% o nā sarcomas palupalu āpau.

ʻOi aku ka maʻamau o kēia maʻi kanesa i nā pākeke ma waena o 20 a me 50 mau makahiki. Eia nō naʻe, hiki i nā keiki ʻōpio ke loaʻa i kēia maʻi kanesa ʻili. Hiki i kekahi mau pēpē ke loaʻa iā DFSP i ka wā hānau. Ua hōʻike ʻia e ka noiʻi ʻoi aku ka nui o ka poʻe ʻeleʻele e loaʻa iā ia ma mua o ka poʻe o nā lāhui ʻē aʻe.

He aha nā ʻano nui o nā DFSP?

Hiki i nā pathologists, nā kauka e nānā i nā ʻiʻo o ke kino ma lalo o kahi microscope, ke haʻi pololei iā ʻoe i ke ʻano o DFSP āu ma ka nānā ʻana i nā cell. Aia kekahi mau ʻano nui:

• ʻO nā puʻupuʻu Bednar (ʻike ʻia hoʻi ʻo DFSP pigmented): Loaʻa i kēia ʻano cell ka nui o ka melanin , ka pigment e hāʻawi i ka ʻili i kona kala. ʻO ia ke kumu e hiki ai i kēia mau puʻupuʻu ke ʻike ʻia i nā kala like ʻole, me ka ʻulaʻula, brown, uliuli, a me ka poni. ʻO kēia ʻano no kahi 5% o nā maʻi DFSP.
• ʻO ka fibroblastoma cell nui: Hōʻike ʻia kēia ʻano e ke alo o nā cell nui. Ua kapa ʻia kēia ʻano he juvenile DFSP no ka mea ʻike pinepine ʻia ia i nā keiki a me nā ʻōpio.
• ʻO Fibrosarcomatous dermatofibrosarcoma protuberans (DFSP-FS): E like me ka mea i ʻōlelo ʻia ma mua, he ʻano sarcoma palupalu koʻikoʻi kēia a laha wikiwiki.
• ʻO Myxoid DFSPs: Hana ʻia kēia me kahi ʻano ʻokoʻa o ka ʻiʻo hoʻopili i kapa ʻia ʻo myxoid stroma. He mea kākaʻikahi loa kēia ʻano DFSP.

He aha ke kumu o DFSP?

ʻO ka mea kupanaha, 9 o 10 mau kānaka e loaʻa ana i ka DFSP he mutation genetic e hoʻoulu ai i ke ʻano . Hana ʻia kēia mutation gene i loko o kā mākou mau cell ma hope o ko mākou hānau ʻana. ʻO ke ʻano kēia, ʻaʻole i hoʻokumu ʻia ka DFSP e kahi kīnā genetic i hoʻoilina ʻia mai ko mākou mau mākua.

He aha nā kumu pilikia no ka hoʻomohala ʻana i ka DFSP?

Ma waho aʻe o ka lāhui, ʻo ka loaʻa ʻana o kekahi ʻano ʻeha ʻili a i ʻole ka ʻōpala hiki ke hoʻonui i ka pilikia o ka hoʻomohala ʻana i ka DFSP. Aia kekahi mau ala e hiki ai ke hōʻeha ʻia ka ʻili:

• Nā kuni
• Ke hana nei i ka radiation therapy
• Nā ʻoki ʻoki
• Nā peʻa

E noʻonoʻo, ua loaʻa iā ʻoe kahi kuni nui a me kahi ʻeha nui. A i ʻole, ua loaʻa iā ʻoe ka lāʻau lapaʻau radiation no kekahi maʻi ʻē aʻe. Ke hōʻino ʻia ka ʻili e nā mea e like me kēia, aia kahi manawa liʻiliʻi e ulu ai kahi maʻi e like me DFSP i ka wā e hiki mai ana, ʻoiai he kakaikahi. ʻO ia ke kumu e makaʻala mau ai mākou i nā loli i ka ʻili.

He aha nā hōʻailona o DFSP?

He palupalu loa nā hōʻailona mua o DFSP a ʻaʻole paha mākou e ʻike iā lākou a ʻike paha iā lākou. Hoʻomaka pinepine kēia mau puʻupuʻu ma ka umauma, ke kua, nā poʻohiwi, ka ʻōpū, a i ʻole nā ​​puʻupuʻu. Hiki iā lākou ke ulu ma nā lima, nā wāwae, ke poʻo, a ma loko o ka waha.

I ka wā mua, ʻike paha ʻoe i kahi wahi liʻiliʻi e like me ka palapū ma ka ʻili.He pālahalaha a ʻeha ʻole. Hiki ke ʻano ʻino iki ke pā aku, a hiki ke loli iki ke kala.

I nā pēpē a me nā kamaliʻi ʻōpio, hiki i ka DFSP ke like me kahi hōʻailona hānau . ʻO ka laulā o kēia mau kiko he hapalua ʻīniha a ʻelua ʻīniha (1 a 5 kenimika).

ʻIke ʻia nā hōʻailona o DFSP i ka ulu ʻana o ke kanesa. I ka ulu ʻana o ka puʻupuʻu, hoʻokomo ia i loko o ka papa luna o ka ʻili (epidermis). Hoʻoulu kēia i nā puʻupuʻu paʻakikī (nodules) e ʻike ʻia ma ka ʻili o ka ʻili . ʻO ke ʻano o ka huaʻōlelo "protuberance" ʻo ia hoʻi "protruding."

Hiki ke ʻike ʻia kēia mau hōʻailona ma nā puʻupuʻu:

• Hiki ke māwae a kahe ke koko me ka maʻalahi.
• Ua pili pono i ka ʻili (ʻaʻole hiki ke hoʻoneʻe ʻia).
• I kou wā ʻelemakule, e ʻō aku ana kou ʻili.
• He paʻakikī ke hoʻopā aku, a i ʻole e like me ka laholio.
• Hiki ke ʻulaʻula-ʻeleʻele, poni, uliuli, a ʻulaʻula paha ke kala.
• He palupalu iki paha ke hoʻopā ʻia.

Aia kekahi mau kūlana ʻē aʻe e hoʻoulu ai i nā hōʻailona like me DFSP?

ʻAe, aia kekahi maʻi ʻili ʻaʻole he maʻi kanesa i kapa ʻia ʻo cellular dermatofibroma . Hiki i kēia ke like loa me DFSP, ʻoiai i kona mau wā mua. ʻO nā dermatofibromas cellular he mau puʻupuʻu palupalu maikaʻi. ʻIke pinepine ʻia lākou ma nā wāwae. I kekahi manawa hiki iā lākou ke ʻānai a ʻeha. ʻAʻole pono ka hapa nui o nā dermatofibromas i ka mālama ʻana. Eia nō naʻe, he mea naʻauao ke ʻole e huikau i nā mea ʻelua, no laila he mea naʻauao ke ʻike i ke kauka inā ʻike ʻoe i kekahi mea hou ma kou ʻili.

Pehea e ʻike ʻia ai ka DFSP (Dermatofibrosarcoma Protuberans)?

Ua ʻike ʻia ka DFSP e nā kauka ʻili , he mau kauka i loaʻa ka hoʻomaʻamaʻa kūikawā i ka maʻi ʻili a me nā maʻi ʻili. E lawe mua kāu kauka i kahi laʻana liʻiliʻi o ka puʻupuʻu ʻili a i ʻole ka kiko a hoʻouna aku i kahi keʻena hoʻokolohua no ka hoʻāʻo ʻana (kahi biopsy ʻili) . Hiki ke wehe ʻia ka puʻupuʻu holoʻokoʻa a hoʻouna ʻia i kahi keʻena hoʻokolohua. A laila e nānā nā pathologists i ka ʻiʻo ma lalo o kahi microscope e ʻike inā he mau hunaola kanesa.

Inā hōʻoia ka biopsy ʻili he DFSP kou, a laila e noi ʻia paha ʻoe e hana i kahi scan MRI . E kōkua kēia i ka hoʻoholo ʻana i ka nui pololei o ka puʻupuʻu a me ka hohonu o kona pālahalaha ʻana i loko o ka ʻili. He mea nui loa kēia ʻike no ka hoʻolālā ʻana i ka mālama ʻana.

He aha nā lāʻau lapaʻau no ka DFSP (Dermatofibrosarcoma Protuberans)?

ʻO ka lāʻau lapaʻau nui a maikaʻi loa no DFSP, ʻo ia ka wehe ʻana i ka puʻupuʻu ma ke ʻoki ʻana. Hoʻohana pinepine nā kauka ʻili i kahi kaʻina hana kūikawā i kapa ʻia ʻo ke ʻoki ʻana ʻo Mohs e wehe i nā puʻupuʻu DFSP. Eia ka mea e hana ʻia i ka wā o kēia ʻoki ʻana:

• ʻO ka mea mua, hoʻokomo ʻia kahi anesthetic kūloko i loko o ka wahi i mālama ʻia, no laila ʻaʻole ʻoe e ʻeha.
• A laila, ʻoki ke kauka a wehe i ka puʻupuʻu kanesa. Akā, ʻaʻole wale ka puʻupuʻu, akā, he wahi liʻiliʻi hoʻi o ka ʻili olakino a puni. Ua kapa ʻia kēia ʻo ka palena .
• ʻO ka mea aʻe, ua nānā ʻia nā kihi (nā palena) o ka ʻiʻo i wehe ʻia ma lalo o kahi microscope e ʻike ai inā aia kekahi mau hunaola kanesa.
• Inā aia nō nā hunaola kanesa ma nā kihi, e wehe ke kauka i kekahi mau ʻiʻo hou aʻe mai ka wahi i hana hou ʻia ai ke ʻoki kino.
• Ma kēia ʻano, hoʻomau ka wehe ʻana i nā ʻiʻo a hiki i ka loaʻa ʻole ʻana o nā hunaola kanesa ma nā kaʻe.
• Ma hope o ka wehe loa ʻia ʻana o ke kanesa, hana ʻia ke ʻoki hana hou inā pono.

Aia kekahi mau lāʻau lapaʻau ʻē aʻe no DFSP me ke ʻoki ʻole ʻana?

ʻOiai inā e wehe ʻia nā puʻupuʻu DFSP ma ke ʻoki ʻana, hiki iā lākou ke hoʻi hou mai i kekahi manawa. Ma hope o kahi ʻoki ākea maʻamau, aia he 20% a 30% ka manawa e hoʻi hou mai ai lākou i loko o ʻekolu mau makahiki. Ma hope o ke ʻoki ʻana ʻo Mohs, ua hoʻemi ʻia kēia pilikia i 4% a 5%. Eia nō naʻe, he mea nui e makaʻala, no ka mea, hiki i nā puʻupuʻu ke hoʻi hou mai ma hope o 10 mau makahiki a ʻoi aku paha.

I kekahi manawa, inā ua pālahalaha ke kanesa i nā ʻāpana ʻē aʻe o ke kino (metastatic DFSP), a inā nui loa ka puʻupuʻu a i ʻole ua kūloko loa e wehe ʻia ma ke ʻoki ʻana, hoʻohana nā kauka i kahi lāʻau i kapa ʻia ʻo Imatinib . Hiki i kēia lāʻau ke hoʻēmi i ka puʻupuʻu, e maʻalahi ai ka wehe ʻana ma ke ʻoki ʻana.

Eia kekahi, inā ʻaʻole hiki i ke ʻoki kino ke hoʻopau loa i ke kanesa, a inā ʻaʻole hiki ke ʻoki kino, hiki ke hoʻohana ʻia ka radiation therapy .

He aha ka manaʻolana no kekahi me DFSP?

He nūhou hōʻoluʻolu maoli kēia. ʻOiai he maʻi kanesa lohi ka ulu ʻana o DFSP, me ka mālama pono ʻana, ʻoi aku ma mua o 99% o ka poʻe e ola no 10 mau makahiki a ʻoi aku paha. No laila, ʻo ka mea nui loa, ʻaʻole e hopohopo a hahai i ka ʻōlelo aʻo a kāu kauka.

He aha kaʻu e hana ai e hoʻomaikaʻi i koʻu ʻike?

Aia kekahi mau mea hiki iā ʻoe ke hana e hoʻomaikaʻi i kou ʻike:

• E hana i nā hoʻokolokolo ʻili maʻamau. E nānā i nā loli hou, nā kiko, a i ʻole nā ​​puʻupuʻu ma kou ʻili. Inā ʻike ʻoe i kekahi mea, e haʻi koke i kāu kauka.
• Ma hope o ka lāʻau lapaʻau, e ʻike i kāu kauka no ka nānā ʻana i ka ʻili ma ka liʻiliʻi i kēlā me kēia ʻekolu a ʻeono mahina no nā makahiki mua ʻekolu. A laila e hele no nā nānā ʻili i hoʻokahi manawa i ka makahiki, a i ʻole e like me ke kuhikuhi a kāu kauka.
• E hōʻemi i ka ʻike ʻana i ka lā. Hoʻonui nā kukuna UV mai ka lā i ka pilikia o nā maʻi ʻili ʻē aʻe. No laila, e kau i ka sunscreen ke hele ʻoe i waho i ka lā, e kau i nā lole pale , a e kaupalena i kou manawa ma waho i ka wā wela loa o ka lā.

Āhea wau e ʻike ai i ke kauka?

Inā ʻike ʻoe i kekahi mau loli hou i kou ʻili, ʻoiai inā loaʻa iā ʻoe kekahi o kēia mau mea, e ʻike pono i ke kauka:

• Nā puʻupuʻu a i ʻole nā ​​kiko hou a mau loa, a i ʻole nā ​​​​​​ulu ʻili hou.
• Nā loli i nā ʻōpuʻu, nā māka hānau, nā ʻeha kahiko, a i ʻole nā ​​peʻa.
• Inā e kahe maʻalahi ke koko o nā puʻupuʻu ma ka ʻili.

E hoʻomanaʻo, ʻaʻole hiki i nā loli ʻili ke hoʻopilikia pinepine. Eia nō naʻe, hiki i kekahi mau loli ke lilo i hōʻailona o ka maʻi ʻili. No laila, inā he kānalua kāu, ʻoi aku ka maikaʻi o ke kipa ʻana i ke kauka e hoʻoponopono iā ia.

He aha kaʻu e nīnau aku ai i kaʻu kauka?

Inā ua ʻike ʻia ʻoe me DFSP, makemake paha ʻoe e nīnau i kāu kauka i nā nīnau e like me:

• No ke aha i loaʻa ai iaʻu kēia maʻi ʻili?
• Aia anei au i ka pilikia o ka loaʻa ʻana o nā ʻano maʻi ʻili ʻē aʻe?
• He aha ka lāʻau lapaʻau maikaʻi loa noʻu?
• Aia kekahi pilikia o ka laha ʻana o kēia maʻi kanesa i nā ʻāpana ʻē aʻe o ke kino?
• ʻEhia pinepine e pono ai iaʻu ke hele mai no nā hoʻokolohua maʻi kanesa?

ʻO ka ʻike ʻana i nā pane i kēia mau nīnau e kōkua iā ʻoe e hoʻomaopopo maikaʻi i ke kūlana a hiki ke kōkua iā ʻoe e wehewehe i kāu mālama ʻana a me nā hoʻolālā e hiki mai ana.

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai

ʻOiai he maʻi ʻili kakaikahi ka DFSP, ʻaʻole paha e laha aku i nā wahi ʻē aʻe o ke kino. Eia nō naʻe, he mea nui ka mālama ʻoki kino e hoʻōki i ka ulu ʻana. Hiki i ka ʻike mua ʻana a me ka mālama kūpono ke hoʻomaikaʻi nui i kou ʻano. No laila, e makaʻala i kou ʻili a e ʻike i ke kauka inā ʻike ʻoe i kekahi mea ʻē. ʻAʻole ʻoe hoʻokahi, a aia nā kauka e hiki ke kōkua iā ʻoe ma kēia huakaʻi.

ʻO DFSP, maʻi ʻili, maʻi ʻili, puʻupuʻu ʻili, ʻoki kino Mohs, nā hōʻailona maʻi ʻili, nā maʻi ʻili