He aha ka DSRCT? Nā Hōʻailona, Nā Kumu, a me ka Lapaʻau

ʻO DSRCT ka pōkole no Desmoplastic Small Round Cell Tumor, kahi ʻano maʻi ʻaʻai kānalua a hoʻouluulu ikaika hoʻi e pili nui ana i nā ʻōpio. Hoʻomaka pinepine kēia maʻi ʻaʻai maʻamau ʻole i loko o ka ʻōpū, ʻoi aku hoʻi i loko o ka peritoneum (ka papa o ka lua o ka ʻōpū), ʻoiai hiki ke ʻike ʻia i nā wahi ʻē aʻe o ke kino.

ʻOiai he maʻamau ʻole ka DSRCT, e pili ana i ka emi ma mua o 200 mau kānaka ma ka honua holoʻokoʻa i kēlā me kēia makahiki, hiki i ka hoʻomaopopo ʻana i kēia kūlana ke kōkua iā ʻoe e ʻike i nā hōʻailona hiki ke loaʻa a ʻike i ka wā e ʻimi ai i ka mālama lapaʻau. ʻOi aku ka nui o nā hihia i nā ʻōpio a me nā ʻōpio, me nā kāne e pilikia ana i ka ʻehā mau manawa ma mua o nā wahine.

He aha ka DSRCT?

ʻO DSRCT kahi sarcoma ʻiʻo palupalu e pili ana i kahi hui o nā maʻi ʻaʻai i kapa ʻia ʻo nā maʻi ʻaʻai pōʻai liʻiliʻi. Loaʻa i ka maʻi ʻaʻai kona inoa mai nā ʻano ʻelua nui: loaʻa iā ia nā sela maʻi ʻaʻai liʻiliʻi, puni, a ua puni ʻia e ka ʻiʻo fibrous mānoanoa i kapa ʻia ʻo desmoplastic stroma.

Hoʻomaka pinepine kēia maʻi ʻaʻai e like me nā ʻāpana he nui ma loko o ka lua o ka ʻōpū ma mua o kahi maʻi ʻaʻai hoʻokahi. Hiki i nā ʻāpana ke ʻokoʻa i ka nui a hoʻolaha pinepine ma nā ʻili peritoneal, ʻo ia ke kumu i kapa ʻia ai ʻo ia he "peritoneal sarcomatosis."

ʻO ka mea e hoʻokaʻawale ai i ka DSRCT, ʻo ia kona ʻano genetic kūikawā. Loaʻa i nā sela maʻi ʻaʻai kahi translocation chromosomal hiʻohiʻona e hana ana i kahi protein fusion maʻamau ʻole, kahi e hoʻokele ai i ka ulu ʻana o ka maʻi ʻaʻai a me ka hana ʻino.

He aha nā hōʻailona o ka DSRCT?

Hiki i nā hōʻailona mua o ka DSRCT ke maʻalahi loa a hoʻomohala mālie paha i nā hebedoma a i ʻole nā mahina. Hoʻomaopopo pinepine nā kānaka i kēia mau hōʻailona ma ke ʻano he mau pilikia digestive liʻiliʻi a i ʻole nā pilikia pilikia.

ʻO nā hōʻailona maʻamau āu e ʻike ai:

• ʻEha ʻōpū mau a i ʻole ka ʻoluʻolu ʻole e hele ʻole me ka hoʻomaha
• Hoʻonui ʻia ka ʻōpū a i ʻole ka bloating e hoʻopilikia ana i ka manawa
• Kahi manaʻo piha ʻana a hiki ma hope o ka ʻai ʻana i nā mea liʻiliʻi
• Kaumaha kaumaha wehe ʻole ʻia ʻoiai ka ʻai maʻamau a i ʻole ka hoʻonui ʻia
• Nausea a me ka luaʻi, ʻoi aku hoʻi ma hope o nā meaʻai
• Loli i nā maʻamau o ka ʻōpū, me ka constipation a i ʻole ka pilikia e hele ai i kahi stool
• Luhi ʻole e hoʻomaikaʻi me ka hoʻomaha
• Nalo o ka makemake

I nā hihia holomua, hiki iā ʻoe ke ʻike i kahi ʻāpana palpable i loko o kou ʻōpū hiki iā ʻoe ke ʻike ma o kou ʻili. ʻIke kekahi poʻe i ka pōkole o ka hanu inā hōʻiliʻili ka wai i loko o ka lua o ka ʻōpū, kahi kūlana i kapa ʻia ʻo ascites.

He mea nui e hoʻomanaʻo e hiki ke loaʻa kēia mau hōʻailona me nā kūlana like ʻole, ʻoi aku ka nui o nā mea maʻamau a emi iki ka pilikia ma mua o ka DSRCT. Eia naʻe, inā ʻoe e ʻike ana i kekahi o kēia mau hōʻailona mau, he mea pono ke kūkākūkā me kāu mea mālama ola.

He aha nā kumu o ka DSRCT?

ʻAʻole ʻike nui ʻia ke kumu pololei o ka DSRCT, hiki ke manaʻo huhū ke ho'āʻo nei ʻoe e hoʻomaopopo i ke kumu e hoʻomohala ai kēia maʻi ʻaʻai. ʻO ka mea a mākou e ʻike nei, ʻo ka hopena o ka DSRCT mai kahi loli genetic kūikawā e hana kūpono ʻole i kekahi mau sela.

ʻO kēia loli genetic kahi translocation ma waena o nā chromosomes 11 a me 22, e hana ana i kahi gene fusion maʻamau ʻole i kapa ʻia ʻo EWSR1-WT1. Hana kēia gene fusion i kahi protein e hoʻopilikia ana i ka ulu ʻana o nā sela maʻamau a me ka māhele ʻana, e alakaʻi ana i ka hoʻomohala ʻana o nā sela maʻi ʻaʻai.

ʻAʻole like me kekahi mau maʻi ʻaʻai ʻē aʻe, ʻaʻole i pili ka DSRCT i:

• Nā kūlana genetic hoʻoilina a i ʻole ka mōʻaukala ʻohana
• Nā hōʻike ʻana i ke kaiapuni a i ʻole nā kumu ola
• Nā mālama radiation a i ʻole chemotherapy ma mua
• Nā maʻi viral
• ʻAi a i ʻole nā maʻamau hoʻoikaika kino

ʻIke ʻia ka loli genetic e hoʻokumu ana i ka DSRCT he hanana kūpono ʻole e hana ana i ka wā o ka māhele ʻana o nā sela. ʻO ia hoʻi, ʻaʻole ia he mea hiki ke pale ʻia ka hoʻomohala ʻana o ka DSRCT ma o nā koho a i ʻole nā hana like ʻole.

I ka wā e ʻike ai i ke kauka no nā hōʻailona DSRCT?

Pono ʻoe e noʻonoʻo e ʻike i kāu kauka inā ʻoe e ʻike ana i nā hōʻailona ʻōpū mau e lōʻihi ana ma mua o ʻelua mau pule, ʻoi aku hoʻi inā e hoʻopilikia ana lākou. ʻOiai he nui loa kēia mau hōʻailona e hoʻokumu ʻia e nā kūlana maʻamau, ʻoi aku ka maikaʻi o ka loiloi ʻana iā lākou.

E ʻimi i ka mālama lapaʻau wikiwiki inā ʻoe e ʻike ana i:

• ʻEha ʻōpū nui, koke
• Hoʻonui wikiwiki ʻia ka ʻōpū ma mua o nā lā ma mua o nā pule
• Luaʻi e pale aku iā ʻoe mai ka mālama ʻana i ka meaʻai a i ʻole nā wai
• Nā hōʻailona o ka pilikia o ka ʻōpū, e like me ka hiki ʻole ke hele i ka gas a i ʻole nā neʻe o ka ʻōpū
• Kaumaha kaumaha wehe ʻole ʻia (ma mua o 10 paona me ka ho'āʻo ʻole)
• Pilikia hanu me ka ʻōpū ʻōpū

E hoʻomanaʻo e aia kāu mea mālama ola ma laila e kōkua iā ʻoe e hoʻokaʻawale i nā hōʻailona hopohopo. Hiki iā lākou ke hana i nā ho'āʻo kūpono e hoʻoholo i ke kumu o kāu mau hōʻailona a hāʻawi iā ʻoe i ka papahana mālama kūpono.

He aha nā pilikia pilikia no ka DSRCT?

Loaʻa i ka DSRCT nā pilikia he nui, ʻo ka mea nui no ke ʻano o kona ulu ʻana a me ka hoʻolaha ʻana ma loko o ka lua o ka ʻōpū. Hiki i ka hoʻomaopopo ʻana i kēia mau pilikia hiki ke kōkua iā ʻoe e ʻike i ka wā e hoʻopilikia ai nā hōʻailona.

ʻO nā pilikia maʻamau:

• Pilikia ʻōpū: Hiki i nā maʻi ʻaʻai ke pani i nā ʻōpū, e pale ana i ka hoʻoheheʻe maʻamau
• Ascites: Hōʻiliʻili wai i loko o ka lua o ka ʻōpū e hoʻokumu ana i ka pehu a me ka ʻoluʻolu ʻole
• Malnutrition: Ma muli o ka makemake ʻole, nausea, a me ka pilikia o ka ʻai ʻana
• Nā clots koko: Hiki i ka maʻi ʻaʻai ke hoʻonui i ka pilikia o nā clots e hana ana i nā moku koko
• Nā pilikia o ka puʻupaʻa: Inā e hoʻopili a komo paha nā maʻi ʻaʻai i ka ʻōnaehana mimi

I nā hihia holomua, hiki i ka DSRCT ke hoʻolaha ma mua o ka lua o ka ʻōpū i nā ʻāpana ʻē aʻe, ʻoi aku ka nui o ka ate, nā māmā, a i ʻole nā lymph nodes. Eia naʻe, he mea maʻamau ʻole kēia ʻano hoʻolaha mamao ma mua o ka hoʻolaha kūloko i loko o ka ʻōpū.

He mea nui e ʻike he hiki i ka mālama kākoʻo hou ke hoʻokele pono i ka nui o kēia mau pilikia, e kōkua ana i ka mālama ʻana i ka maikaʻi o ke ola i ka wā o ka mālama ʻana. E nānā kāu hui lapaʻau no kēia mau pilikia a e hoʻoponopono koke inā loaʻa lākou.

Pehea e ʻike ʻia ai ka DSRCT?

ʻO ka ʻike ʻana i ka DSRCT e pili pinepine ana i nā ʻanuʻu he nui, ʻoiai pono nā kauka e kāpae i nā kūlana maʻamau mua. Hoʻomaka pinepine ke kaʻina hana me kāu mōʻaukala lapaʻau a me kahi hoʻokolokolo kino o kou ʻōpū.

E kauoha paha kāu kauka i nā ho'āʻo kiʻi e kiʻi i kahi hiʻohiʻona maikaʻi o ka mea e hana ana i loko o kou ʻōpū. ʻO ka CT scan o ka ʻōpū a me ka pelvis ka hoʻokolohua kiʻi mua pinepine e hana ʻia, ʻoiai hiki iā ia ke hōʻike i ka nui, kahi, a me ka nui o nā ʻāpana i loaʻa.

Hiki i nā ho'āʻo hou ke komo pū:

• Nā MRI scan no nā kiʻi kikoʻī o nā ʻiʻo palupalu
• Nā PET scan e nānā no ka hana maʻi ʻaʻai ma o ke kino
• Nā ho'āʻo koko e nānā i kou olakino holoʻokoʻa a me ka hana o nā ʻāpana
• Nā hōʻailona maʻi ʻaʻai, ʻoiai ʻaʻole kēia mau mea kūikawā no ka DSRCT

Pono ka ʻike pololei i kahi biopsy, kahi e wehe ʻia ai kahi ʻāpana liʻiliʻi o ka ʻiʻo a nānā ʻia ma lalo o ka microscope. E ʻimi ka pathologist i nā sela puni liʻiliʻi a e hana i nā ho'āʻo kūikawā e hōʻoia i ka fusion gene EWSR1-WT1 e hoʻomaopopo ana i ka DSRCT.

Hiki i kēia kaʻina hana diagnostic ke lawe i nā lā a i ʻole nā pule, hiki ke manaʻo kaumaha. E hoʻomanaʻo e hōʻoia kēia ala hoʻokolokolo piha iā ʻoe e loaʻa ka ʻike pololei loa, he mea nui ia no ka hoʻolālā ʻana i ka hoʻolālā mālama maikaʻi loa.

He aha ka mālama no ka DSRCT?

Hoʻopili pinepine ka mālama ʻana i ka DSRCT i kahi ala hana he nui e hui pū ana i nā ʻano therapy like ʻole. ʻO ka pahuhopu, ʻo ia ke hoʻemi i nā maʻi ʻaʻai i ka hiki a hoʻomalu i ka maʻi no ka manawa lōʻihi.

ʻO ke ala mālama maʻamau e komo pū ana:

1. Chemotherapy: Nā pōʻai he nui o nā lāʻau anti-cancer ikaika i hāʻawi ʻia ma luna o nā mahina he nui
2. ʻOihana: Wehe ʻana i ka nui o ka maʻi ʻaʻai i hiki, hoʻohui pinepine ʻia me ka chemotherapy wela pololei i loko o ka ʻōpū
3. Radiation therapy: Nā kukuna ikehu kiʻekiʻe i hoʻonohonoho ʻia i nā sela maʻi ʻaʻai i koe

Hoʻomaka pinepine ka papa chemotherapy a hiki ke lōʻihi i 4-6 mau mahina. ʻO nā hui lāʻau maʻamau e komo pū ana me ifosfamide, carboplatin, etoposide, a me doxorubicin. Hana kēia mau lāʻau ma o ka hoʻopili ʻana i nā sela maʻi ʻaʻai e māhele wikiwiki ana.

ʻO ka ʻoki kino, ke hiki, e komo pū ana i kahi kaʻina hana i kapa ʻia ʻo cytoreductive surgery me hyperthermic intraperitoneal chemotherapy (HIPEC). Hoʻopili kēia i ka wehe ʻana i nā maʻi ʻaʻai i ʻike ʻia a laila holoi ʻana i ka lua o ka ʻōpū me nā lāʻau chemotherapy wela.

I ka wā o ka mālama ʻana, e kaulike pū kāu hui lapaʻau i ka mālama kākoʻo e kōkua i ka hoʻokele ʻana i nā hopena a mālama i kou ikaika a me ka maikaʻi o ke ola. Hiki paha kēia ke komo pū me nā lāʻau no ka nausea, kākoʻo meaʻai, a me nā mālama e pale aku i nā maʻi.

Pehea e hoʻokele ai i nā hōʻailona ma ka home i ka wā o ka mālama ʻana i ka DSRCT?

Hiki i ka hoʻokele ʻana i nā hōʻailona ma ka home ke kōkua iā ʻoe e ʻoluʻolu a mālama i kou ikaika i ka wā o ka mālama ʻana. Hiki i nā hoʻoponopono liʻiliʻi i kēlā me kēia lā ke hoʻolilo i kahi ʻokoʻa nui i kou manaʻo holoʻokoʻa.

No nā hōʻailona digestive, ʻoi aku ka maikaʻi o ka ʻai ʻana i nā meaʻai liʻiliʻi, pinepine ma mua o ka ho'āʻo ʻana e ʻai i nā ʻāpana nui. E kaulike i nā meaʻai maʻalahi ke hoʻoheheʻe a hoihoi iā ʻoe, ʻoiai inā ʻaʻole maikaʻi kou mau punahele i kēia manawa.

No ka hoʻokele ʻana i ka luhi:

• E hoʻomaha ke pono ʻoe, akā e ho'āʻo e noho mālie i ka wā hiki
• E hele i nā hele pōkole a i ʻole e hoʻolōʻihi māmā e like me ka ʻae ʻia
• E mālama i kahi papa moe maʻamau ke hiki
• E noi aku i nā ʻohana a me nā hoaaloha no ke kōkua me nā hana i kēlā me kēia lā

No ka nausea a me nā pilikia makemake, e ho'āʻo e ʻai i nā meaʻai palupalu e like me nā crackers, toast, a i ʻole ka laiki. Hiki i ka tī ginger a i ʻole nā mea hoʻohui ginger ke kōkua me ka nausea. E noho hydrated ma o ka inu ʻana i nā wai liʻiliʻi i ka lā holoʻokoʻa.

E mālama i kāu mau hōʻailona a me nā hopena e hiki ai iā ʻoe ke kūkākūkā me kāu hui mālama ola. Hiki iā lākou ke hoʻoponopono pinepine i nā lāʻau a i ʻole hāʻawi i nā mālama kākoʻo hou e kōkua iā ʻoe e ʻoluʻolu.

Pehea ʻoe e hoʻomākaukau ai no kāu mau hālāwai kauka?

Hiki i ka hoʻomākaukau ʻana no kāu mau hālāwai lapaʻau ke kōkua iā ʻoe e hana i ka nui o kou manawa me kāu hui mālama ola a hōʻoia iā ʻoe e loaʻa nā pane i kāu mau nīnau a pau. He ala lōʻihi ka hoʻomākaukau iki i ke kōkua ʻana iā ʻoe e manaʻo ʻoi aku ka mana o kāu mālama.

Ma mua o kēlā me kēia hālāwai, e kākau i kāu mau hōʻailona o kēia manawa, me ka wā i hoʻomaka ai lākou a pehea lākou i loli ai. E hoʻomaopopo i nā hōʻailona hou a i ʻole nā hopena āu e ʻike nei mai ka mālama ʻana.

E hoʻomākaukau i kahi papa inoa o nā nīnau āu e makemake ai e nīnau:

• He aha ka mea i hōʻike ʻia e kaʻu mau hopena ho'āʻo hou?
• Pehea ka hana o kaʻu mālama?
• He aha nā hopena e pono ai au e nānā?
• Aia nā ala e hoʻokele ai i kaʻu mau hōʻailona o kēia manawa?
• I ka wā hea kaʻu mālama ʻana e hoʻonohonoho ʻia?

E lawe mai i kahi papa inoa piha o nā lāʻau āpau āu e lawe nei, me nā lāʻau over-the-counter a me nā mea hoʻohui. E noʻonoʻo e lawe mai i kahi lālā ʻohana a hoaaloha paha e kōkua iā ʻoe e hoʻomanaʻo i ka mea i kūkākūkā ʻia a hāʻawi i ke kākoʻo manaʻo.

Mai kānalua e noi aku no ka wehewehe inā ʻaʻole ʻoe e hoʻomaopopo i kahi mea. Makemake kāu hui mālama ola e hōʻoia iā ʻoe e hoʻomaopopo piha i kou kūlana a me ka papahana mālama.

He aha ka lawe nui e pili ana i ka DSRCT?

ʻO DSRCT kahi maʻi ʻaʻai kānalua akā koʻikoʻi e pili nui ana i nā ʻōpio. ʻOiai hiki ke manaʻo kaumaha ka ʻike, ua hoʻomaikaʻi nā holomua i ka mālama ʻana i nā hopena no nā mea maʻi he nui i nā makahiki i hala iho nei.

ʻO ka mea nui loa e hoʻomanaʻo, ʻaʻole ʻoe wale nō i kēia huakaʻi. Loaʻa i kāu hui lapaʻau ka ʻike i ka mālama ʻana i kēia maʻi ʻaʻai kānalua a e hana pū me ʻoe e hoʻomohala i ka papahana mālama maikaʻi loa no kāu kūlana kūikawā.

Hiki i ka ʻike mua ʻana i nā hōʻailona a me ka mālama lapaʻau wikiwiki ke hoʻolilo i kahi ʻokoʻa i nā hopena mālama. Inā ʻoe e ʻike ana i nā hōʻailona ʻōpū mau, ʻoi aku hoʻi inā he ʻōpio ʻoe, mai kānalua e kūkākūkā me kāu mea mālama ola.

E hoʻomanaʻo ʻaʻole ʻo ka loaʻa ʻana o nā hōʻailona hopohopo ʻo ia ka DSRCT. He maʻamau loa kēia maʻi ʻaʻai, a ʻoi aku ka nui o kāu mau hōʻailona e hoʻokumu ʻia e kahi kūlana maʻamau, hiki ke mālama ʻia. Eia naʻe, ʻo ka loiloi ʻana iā ʻoe e hāʻawi iā ʻoe i ka maluhia o ka manaʻo a hōʻoia iā ʻoe e loaʻa ka mālama kūpono ʻoiai ke kumu.

Nā nīnau i nīnau pinepine ʻia e pili ana i ka DSRCT

He hereditary anei ka DSRCT?

ʻAʻole, ʻaʻole hereditary ka DSRCT a ʻaʻole holo i nā ʻohana. ʻIke ʻia nā loli genetic e hoʻokumu ana i kēia maʻi ʻaʻai e hana kūpono ʻole i ka wā o ka māhele ʻana o nā sela. ʻAʻole hoʻonui ka loaʻa ʻana o kahi lālā ʻohana me ka DSRCT i kou pilikia o ka hoʻomohala ʻana.

Pehea ka maʻamau ʻole o ka DSRCT?

He maʻamau loa ka DSRCT, me ka emi ma mua o 200 mau hihia hou i ʻike ʻia ma ka honua holoʻokoʻa i kēlā me kēia makahiki. No ka hoʻokomo ʻana i kēia i ka manaʻo, ʻoi aku ka nui o kou pilikia e pā ʻia e ka uila ma mua o ka hoʻomohala ʻana i ka DSRCT. ʻO kēia maʻamau ʻole ke kumu hoʻokahi e hiki ai ke pilikia ke ʻike mua.

Hiki ke ho'ōla ʻia ka DSRCT?

ʻOiai he maʻi ʻaʻai ikaika ka DSRCT, loaʻa i kekahi mau mea maʻi ka remission lōʻihi me ka mālama ikaika. Ua kōkua ka hui pū ʻana o ka chemotherapy, ka ʻoki kino, a me ka radiation therapy i kekahi poʻe e ola me ka maʻi ʻaʻai ʻole no nā makahiki he nui. Ke hoʻomau nei ka hoʻomaikaʻi ʻana i nā hopena mālama ke aʻo hou nā kauka e pili ana i kēia maʻi ʻaʻai kānalua.

He aha ka makahiki maʻamau i ʻike ʻia ai ka DSRCT?

ʻIke pinepine ʻia ka hapa nui o nā kānaka me ka DSRCT ma waena o nā makahiki 10 a me 30, me ka nui o nā hihia i loaʻa i nā makahiki hope o nā makahiki ʻōpio a me nā makahiki iwakālua mua. Eia naʻe, ua hōʻike ʻia nā hihia i nā keiki ʻōpio e like me 5 a me nā mākua kahiko e like me 50, ʻoiai he mea maʻamau ʻole kēia mau mea.

Pehea ka lōʻihi o ka mālama ʻana i ka DSRCT?

Hoʻopau pinepine ke kaʻina hana mālama holoʻokoʻa ma kahi o 12-18 mau mahina, ʻoiai hiki ke ʻokoʻa kēia ma muli o nā kūlana pilikino. Hoʻopili kēia i nā mahina he nui o ka chemotherapy, a laila ka ʻoki kino (inā hiki), a laila ka chemotherapy hou a i ʻole ka radiation therapy. E hāʻawi kāu hui lapaʻau iā ʻoe i kahi manawa kikoʻī e pili ana i kāu papahana mālama.