Tele endocrine neoplasia (MEN) II

Tele endocrine neoplasia, ituaiga II (MEN II) o se faʻamaʻi pasiina i aiga lea o se tasi po o le sili atu o le endocrine glands ua soʻona fai pe fausia ai se tumo. Endocrine glands e masani ona aofia ai aofia ai:

• Adrenal gland (pe a ma le afa taimi)
• Parathyroid gland (20% o le taimi)
• O lou totoa (toetoe o taimi uma)

Tele endocrine neoplasia (MEN I) o se fesoʻotaʻiga tulaga.

O le mafuaʻaga o le MEN II o se faʻaletonu i se kenera taʻua o le RET. Lenei faʻaletonu mafua ai le tele o tumors e aliali mai i le tutusa tagata, ae le tatau i le taimi e tasi.

Faʻaaofiaina o le adrenal gland e masani ona i ai ma le tumo e taʻua o le pheochromocytoma.

O le aʻafia o le tiroida e masani lava ona iai ma le tumo e taʻu ole medullary carcinoma ole thyroid.

Tumua i le tiroida, adrenal, poʻo parathyroid glands ono tupu i le tele o tausaga vavaeʻese.

O le faʻamaʻi ono tupu i soʻo se tausaga, ma aafia tutusa ai aliʻi ma fafine. Ole mafuaʻaga taua o se talaʻaga aiga o MEN II.

E lua subtypes o TAG II. O i latou o MEN IIa ma IIb. MEN IIb e tau le masani ai.

O faʻailoga e ono eseese. Peitai, e tali tutusa ma i latou o:

• Toto kanesa o le thyroid
• Pheochromocytoma
• Parathyroid adenoma
• Parathyroid hyperplasia

Ina ia faʻamaonia le tulaga lea, e vaʻai le tagata tausi soifua maloloina mo se suiga i le RET gene. E mafai ona faia lea ile suʻega ole toto. O faʻataʻitaʻiga faʻaopopo e faia e iloa ai poʻo fea homone o loʻo soʻona gaosia.

O se suʻega faʻaletino e ono faʻaalia:

• Faʻateleina lymph nodes i le ua
• Fiva
• Toto maualuga
• Toto vave fatu
• O lou nifo nodules

Faʻataʻitaʻiga faʻataʻitaʻiga faʻaogaina e faʻailoa ai tumors ono aofia ai:

• Suʻega CT scan
• Ata o fatugaʻo poʻo ureter
• MIBG scintiscan
• MRI o le manava
• Vaʻai faʻasolosolo
• Ultrasound o le tirouro

O suʻega toto e faʻaaogaina e iloa ai le lelei o le galue o nisi manava i le tino. Latou te ono aofia ai:

• Calcitonin tulaga
• Toto alkaline phosphatase
• Toto kalisiu
• Toto parathyroid fua faatatau maualuga
• Fosipolo toto
• Mimi catecholamines
• Metanephrine mimi

O isi suʻega poʻo taualumaga e ono faia e aofia ai:

• Biopsy Adrenal
• Electrocardiogram (ECG)
• Biopsy o lou thyroid

E manaʻomia se taotoga e aveʻese ai le pheochromocytoma, lea e ono lamatia ai le ola ona o homone na te faia.

Mo vailaʻau oona o le thyroid, e tatau ona matua aveʻesea uma le totoa o le totoa (thyroid gland) ma faʻataʻaliʻiga lymph nodes. O le fofoina o le homone homone e faia pe a maeʻa le taotoga.

Afai e iloa le tamaititi na te aveina le fesuiaiga o le gen RET, o le taotoga e aveese ai le tiroida ae e te leʻi oʻo mai e kanesa. Lenei tatau ona talanoaina ma se fomaʻi o ia e masani i lenei tulaga. O le a faia i le amataga o tausaga (i luma tausaga 5) i tagata ma iloa MEN IIa, ma i luma o tausaga 6 masina i tagata ma MEN IIb.

Pheochromocytoma e masani ona le maua i le kanesa (benign). Medullary carcinoma o le tirouro o se kanesa malosi tele ma ono lamatia ai le kanesa, ae o le vave suʻesuʻe ma taotoga e masani ona taitai atu ai i se fofo. O le taʻotoga e le mafai ona togafitia ai le autu o le MEN II.

O le sosolo o sela kanesa o se faigata faigata.

Valaʻau lau tautua pe a e maitauina faʻailoga o le MEN II pe afai e maua se tasi i lou aiga faʻamaʻi.

O le suʻesuʻe vavalalata aiga o tagata ma MEN II mafai ona taitai atu ai i le vave mauaina o le syndrome ma isi kanesa. Lenei mafai faʻatagaina mo laʻasaga e puipuia faʻalavelave.

Sipple syndrome; MEN II; Pheochromocytoma - MEN II; Kanesa o lou thyroid - pheochromocytoma; Kanesa Parathyroid - pheochromocytoma

• Laʻau Endocrine

'Upega Tafaʻilagi National Comprehensive Cancer Network. Taʻiala faʻataʻi mo togafitiga ile oncology (NCCN guideines): neuroendocrine tumors. Faʻamatalaga 1.2019. www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. Faʻafouina Mati 5, 2019. Avanoa ia Mati 8, 2020.

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Nieman LK, Spiegel AM. Faʻafitauli polyglandular. I totonu: Goldman L, Schafer AI, eds. Faʻafomaʻi Goldman-Cecil. 26th lomiga. Filatelefia, PA: Elsevier; 2020: chap 218.

Tacon LJ, Learoyd DL, Robinson BG. Tele endocrine neoplasia ituaiga 2 ma medullary thyroid carcinoma. I totonu: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Matutua ma Tamaiti. 7th ed. Filatelefaia, PA: Elsevier Saunders; 2016: mata 149