ʻO ka neuroblastoma kekahi o nā maʻi maʻamau maʻamau i nā keiki. Ke kamaʻilio nei mākou no ka maʻi maʻi maʻi extra-cerebral no ka mea hoʻomaka ia i loko o ka ʻōnaehana nerve akā ʻaʻole ia i loko o ka lolo. Hiki ke noʻonoʻo ʻia kekahi mau koho lapaʻau ma muli o ka hihia.

Ka wehewehe o neuroblastoma

ʻO ka neuroblastoma kekahi ʻano o ka maʻi kanesa. ʻO kēia maʻi maʻi maʻi ke ʻano o ka hoʻomohala ʻana ma ke kiʻekiʻe o nā neuroblasts, ʻo ia hoʻi nā pūnaʻi aʻalolo ʻoi ʻole o ka ʻōnaehana sympathetic. ʻO ka mea hope kekahi o nā pou ʻekolu o ka ʻōnaehana neʻe autonomic e alakaʻi ana i nā hana involuntary o ke kino e like me ka hanu ʻana a me ka ʻai ʻana.

Hiki ke ulu ka neuroblastoma ma nā wahi like ʻole o ke kino. Eia naʻe, ʻike pinepine ʻia kēia ʻano maʻi maʻi maʻi i loko o ka ʻōpū ma ke kiʻekiʻe o nā kelepa adrenal (aia ma luna o nā puʻupaʻa), a me ke kuamoʻo. ʻOi aku ka kakaikahi, hiki ke loaʻa ma ka ʻāʻī, ka ʻāʻī a i ʻole ke pelvis (pelvis liʻiliʻi).

I kona ulu ʻana, hiki i kahi neuroblastoma ke hana i ka metastasis. ʻO kēia nā maʻi maʻi maʻi lua: pakele nā ​​​​pūnaewele o ka maʻi maʻi mua a hoʻopaʻa i nā ʻiʻo a me / a i ʻole nā ​​ʻāpana ʻē aʻe.

Hoʻokaʻawale i nā neuroblastomes

Hiki ke hoʻokaʻawale ʻia nā maʻi maʻi e like me nā ʻāpana he nui. No ka laʻana, kōkua ka hana ʻana i ka loiloi i ka nui o ka maʻi kanesa. I ka hihia o neuroblastoma, hoʻohana ʻia nā ʻano ʻelua o ka staging.

ʻO ka pae mua ka mea i hoʻohana pinepine ʻia. Hoʻokaʻawale ia i nā neuroblastomas mai ka pae 1 a hiki i ka 4 a loaʻa pū kekahi i kahi pae 4. ʻO kēia ka hoʻokaʻawale ʻana ma muli o ke koʻikoʻi, mai ka liʻiliʻi liʻiliʻi a hiki i ke koʻikoʻi loa:

• ʻO nā pae 1 a hiki i ka 3 e pili ana i nā ʻano kūloko;
• ʻO ka pae 4 e koho i nā ʻano metastatic (ka neʻe ʻana o nā pūnaʻi maʻi maʻi a me ka noho ʻana o nā hale ʻē aʻe o ke kino);
• ʻO ka pae 4s kahi ʻano kikoʻī i hōʻike ʻia e nā metastases i loko o ke ake, ʻili a me ka iwi iwi.

ʻO ka papa ʻelua he 4 mau pae: L1, L2, M, MS. ʻAʻole wale ia e hiki ai ke hoʻokaʻawale i ka localized (L) mai nā ʻano metastatic (M), akā e noʻonoʻo pū kekahi i kekahi mau mea pilikia.

Ke kumu o ka neuroblastome

E like me nā ʻano maʻi maʻi ʻē aʻe, ʻaʻole i hoʻokumu piha ʻia nā neuroblastomas.

I kēia lā, ua ʻike ʻia ʻo ka hoʻomohala ʻana o kahi neuroblastoma hiki ke kumu a makemake ʻia e nā maʻi like ʻole:

• ʻano 1 neurofibromatosis, a i ʻole ka maʻi o Recklinghausen, kahi mea ʻino i ka hoʻomohala ʻana o nā ʻiʻo neural;
• ʻO ka maʻi o Hirschsprung ka hopena o ka nele o nā ganglia nerve ma ka paia o ka ʻōpū;
• ʻO ka maʻi maʻi Ondine, a i ʻole congenital central alveolar hypoventilation syndrome, kahi i hōʻike ʻia e ka nele o ka mana o ka hanu a me ka pōʻino i ka ʻōnaehana neʻe autonomic.

I nā hihia koʻikoʻi, ua ʻike ʻia ka neuroblastoma i nā poʻe me:

• ʻO ka maʻi Beckwith-Wiedemann kahi i hōʻike ʻia e ka ulu nui a me nā hemahema hānau;
• ʻO ka maʻi Di-George, kahi kīnā hānau i loko o nā chromosomes e hopena maʻamau i nā hemahema o ka naʻau, ka dysmorphism o ka maka, ka lohi o ka ulu ʻana a me ka immunodeficiency.

ʻO ka diagnostic du neuroblastome

Hiki ke kānalua ʻia kēia ʻano maʻi maʻi ma muli o kekahi mau hōʻailona lapaʻau. Hiki ke hōʻoia a hoʻonui ʻia ka ʻike o ka neuroblastoma e:

• he ho'āʻo mimi e loiloi ana i ke kiʻekiʻe o kekahi mau metabolites e piʻi ana ka excretion i ka wā o ka neuroblastoma (eg homovanilic acid (HVA), vanillylmandelic acid (VMA), dopamine);
• ke kiʻi kiʻi o ka maʻi maʻi mua ma ke ultrasound, CT scan a i ʻole MRI (magnetic resonance imaging);
• MIBG (metaiodobenzylguanidine) scintigraphy, e pili ana i kahi ho'āʻo kiʻi i ka lāʻau nuklea;
• kahi biopsy e komo ana i ka lawe ʻana i kahi ʻāpana o ke kiko no ke kālailai ʻana, keu hoʻi inā kānalua ʻia ka maʻi ʻaʻai.

Hiki ke hoʻohana ʻia kēia mau hoʻāʻo e hōʻoia i ka hōʻoia ʻana o kahi neuroblastoma, e ana i kona nui a e nānā i ka hele ʻana a i ʻole ka loaʻa ʻole o nā metastases.

Loaʻa pinepine nā neuroblastoma i nā pēpē a me nā keiki liʻiliʻi. Hōʻike lākou i ka 10% o nā maʻi maʻi maʻi kamaliʻi a me 15% o nā maʻi maʻi maʻi i nā keiki ma lalo o 5 mau makahiki. I kēlā me kēia makahiki, ma kahi o 180 mau hihia hou i ʻike ʻia ma Farani.

• Asymptomatic: Hiki ke ʻike ʻole ʻia kahi neuroblastoma, ʻoi aku hoʻi i kona wā mua. ʻIke pinepine ʻia nā hōʻailona mua o ka neuroblastoma i ka wā e laha ana ka maʻi koko.
• ʻO ka ʻeha kūloko: ʻO ka ulu ʻana o ka neuroblastoma e hele pinepine ʻia me ka ʻeha ma ka wahi i hoʻopilikia ʻia.
• ʻO ka pehu kūloko: Hiki ke ʻike ʻia kahi puʻupuʻu, puʻu, a pehu paha ma ka wahi i hoʻopilikia ʻia.
• Hoʻololi i ke kūlana maʻamau: Hoʻopilikia ka neuroblastoma i ka hana kūpono o ka ʻōnaehana nerve, hiki ke hopena i ka pau ʻana o ka ʻai, ke kaumaha o ke kaumaha, ka ulu mālie.

I kēia lā, hiki ke hoʻokō ʻia ʻekolu mau lāʻau lapaʻau nui:

• ʻoki kino e hemo i ka puʻupuʻu;
• radiation therapy, e hoʻohana ana i ka radiation e luku ai i nā maʻi kanesa;
• ka chemotherapy, kahi e hoʻohana ai i nā kemika e kaupalena i ka ulu ʻana o nā pūnaʻi kanesa.

Ma hope o nā lāʻau lapaʻau i ʻōlelo ʻia ma luna nei, hiki ke hoʻokomo ʻia kahi stem cell transplant e hoʻihoʻi i ka hana kūpono o ke kino.

ʻO ka Immunotherapy kahi ala hou no ka mālama ʻana i ka maʻi kanesa. Hiki ke hoʻokō a i ʻole he mea ʻē aʻe i nā lāʻau lapaʻau i ʻōlelo ʻia ma luna. Nui ka noiʻi e holo nei. ʻO ka pahuhopu o ka immunotherapy ʻo ia ka hoʻoulu ʻana i nā pale pale pale o ke kino e hakakā i ka ulu ʻana o nā maʻi kanesa.

ʻAʻole maopopo ka kumu o nā neuroblastomas a hiki i kēia lā. ʻAʻole hiki ke ʻike ʻia ke ʻano pale.

ʻO ka pale ʻana i nā pilikia pili i ka ʻike mua. I kekahi manawa hiki ke ʻike i kahi neuroblastoma i ka wā o ka ultrasound antenatal. A i ʻole, pono ka makaʻala ma hope o ka hānau ʻana. He mea nui ka nānā 'ana i ke kino o ke keiki.