Neoplasia endocrine maha (MEN) II

ʻO ka neoplasia endocrine maha, ʻano II (MEN II) kahi maʻi i hoʻoili ʻia i waena o nā ʻohana kahi kahi a ʻoi aku paha o nā endocrine glands e hana nui ai a hana ai i kahi tumo. Hoʻopili pinepine nā gland Endocrine i:

• Adrenal gland (ma kahi o ka hapalua o ka manawa)
• Paona parathyroid (20% o ka manawa)
• Kelepaʻi thyroid (ʻaneʻane i nā manawa āpau)

ʻO ka neoplasia endocrine maha (MEN I) kahi ʻano pili.

ʻO ke kumu o MEN II kahi kīnā i kahi moʻokūʻauhau i kapa ʻia ʻo RET. Kuhi kēia kīnā i nā tumors he nui i ka mea like, akā ʻaʻole pono i ka manawa like.

ʻO ka komo pū ʻana o ka adrenal gland ka mea pinepine me kahi tumo i kapa ʻia he pheochromocytoma.

ʻO ka hoʻokomo ʻana i ka gland thyroid ka mea pinepine me kahi tumo i kapa ʻia he carcinoma medullary o ka thyroid.

Hiki i nā tumors i ka thyroid, adrenal, a i ʻole nā ​​parathyroid glands ke kū he mau makahiki i kaawale.

Hiki ke hana i ka maʻi i nā makahiki, a pili like i nā kāne a me nā wahine. ʻO ke kumu pilikia nui kahi moʻolelo ʻohana o MEN II.

ʻElua mau subtypes o MEN II. ʻO MEN IIa lāua ʻo IIb. ʻO MEN IIb ka mea liʻiliʻi loa.

E loli paha nā ʻōuli. Eia nō naʻe, ʻano like lākou i nā ʻano o:

• Ka maʻi kanesa o ka thyroid
• Pheochromocytoma
• Parathyroid adenoma
• ʻO hyperplasia Parathyroid

No ke kuhi ʻana i kēia ʻano, nānā ka mea mālama ola no kahi hoʻololi i ka gen RET. Hiki i kēia ke hana me kahi hōʻike koko. Hana ʻia nā hoʻokolohua hou aʻe e hoʻoholo i nā hormones e hoʻonui ʻia.

Hōʻike kahi hoʻokolohua kino:

• Hoʻonui ʻia nā lymph node i ka ʻāʻī
• Fiva
• Piʻi koko kiʻekiʻe
• Helu puʻuwai wikiwiki
• ʻO nā nodule thyroid

ʻO nā hoʻokolohua kiʻi i hoʻohana ʻia e ʻike ai i nā tumors e hoʻopili pū paha:

• Scanner CT ʻōpū
• Kiʻi o nā puʻupaʻa a i ʻole nā ​​ureter
• MIBG scintiscan
• MRI o ka ʻōpū
• ʻImi thyroid
• Ultrasound o ka thyroid

Hoʻohana ʻia nā hoʻokolohua koko e ʻike i ka maikaʻi o ka hana ʻana o kekahi mau glands i loko o ke kino. Pākuʻi ʻia lākou:

• Pae Calcitonin
• Ka phalphasease alkaline koko
• Pāpā kō
• Pae pae parathyroid ke koko
• Pākuʻi koko
• ʻO nā catecholamines mimi
• Metanephrine mimi

Nā hoʻokolohua ʻē aʻe a i ʻole nā ​​kaʻina hana i hana ʻia e komo pū me:

• ʻO Biopsy adrenal
• Electrocardiogram (ECG)
• ʻO ka biopsy thyroid

Pono ka hana ʻoki e wehe i kahi pheochromocytoma, hiki ke hoʻoweliweli i ke ola ma muli o nā homone i hana ʻia.

No ka maʻi kanesa medroidary o ka thyroid, pono e hemo loa ka pākana thyroid a me nā lymph node e pili ana. Hāʻawi ʻia ka thyroid hormone replacement therapy ma hope o ke kaha ʻana.

Inā ʻike ʻia kahi keiki e hāpai i ka mutation gene RET, noʻonoʻo ʻia ke kaha ʻana e wehe i ka thyroid ma mua o ka lilo ʻana i maʻi ʻaʻa. Kūkākūkā kēia me kahi kauka i kamaʻāina loa i kēia ʻano. E hana ʻia ia i ka wā ʻōpio (ma mua o ka makahiki 5) i ka poʻe me MEN IIa i ʻike ʻia, a ma mua o 6 mau makahiki i ka poʻe me MEN IIb.

ʻAʻole pinepine ʻo Pheochromocytoma i ka maʻi ʻaʻai (benign). ʻO ka maʻi kanesa medullary o ka thyroid he maʻi hōʻeha nui a make paha ia, akā ʻo ka ʻike mua a me ke kaha ʻana ke alakaʻi pinepine i kahi lāʻau. ʻAʻole hiki i ke ʻoki kino ke hoʻōla i ke kumu o MEN II.

ʻO ka hoʻolaha ʻana o nā hunaola maʻi ʻaʻai he komikino hiki.

Kāhea i kāu mea hāʻawi inā ʻike ʻoe i nā ʻōuli o MEN II a i ʻole inā loaʻa i kekahi o kou ʻohana kēlā ʻano hōʻailona.

ʻO ka ʻimi ʻana i nā ʻohana pili o nā kānaka me MEN II i hiki ke alakaʻi i ka ʻike mua ʻana i ka maʻi maʻi a me nā maʻi ʻino e pili ana. ʻAe paha kēia i nā ʻanuʻu e pale ai i nā pilikia.

Sipple maʻi maʻi; MEN II; Pheochromocytoma - MEN II; Ka maʻi ʻaʻai thyroid - pheochromocytoma; Ka maʻi ʻaʻai Parathyroid - pheochromocytoma

• Nānā Endocrine

Pūnaewele Pūnaewele Manuahi Manuahi. Nā kulekele hana lapaʻau i ka oncology (NCCN guideine): neuroendocrine tumors. Mana 1.2019. www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. Hoʻohou ʻia Malaki 5, 2019. Loaʻa ʻia ma Malaki 8, 2020.

Newey PJ, Thakker RV. Neoplasia endocrine lehulehu. I: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, eds. ʻO Williams Textbook of Endocrinology. 14th ed. Piladelapia, PA: Elsevier; 2020: chap 42.

Nieman LK, Spiegel AM. Nā maʻi Polyglandular. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 218.

ʻO Tacon LJ, Learoyd DL, Robinson BG. ʻO ka neoplasia endocrine nui 2 a me ka carroidoma thyroid medullary. I: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Makua a Pediatric. ʻEpekema 7 Piladelapia, PA: Elsevier Saunders; 2016: chap 149.