Nevoid basal cell kanesa kanesa

Nevoid basal cell carcinoma syndrome o se vaega o faʻaletonu pasi mai i aiga. O le faʻamaʻi e aofia ai le paʻu, neula, mata, endocrine glands, urinary ma reproductive system, ma ponaivi.

E mafua ai le le masani ai foliga vaaia ma le maualuga lamatiaga mo paʻu kanesa ma noncancerous tumors.

Nevoid basal cell carcinoma nevus syndrome o se tulaga seasea tupuʻaga. O le vaega autu e fesoʻotaʻi ma le faʻamaʻi e lauiloa o le PTCH ("patched"). O le lona lua gafa, ua taʻua SUFU, na fesoʻotaʻi foʻi ma lenei tulaga.

O mea le masani ai i nei ituaiga o genes e masani ona pasi ane i totonu o aiga o se uiga autosomal. O lona uiga oe faʻatupuina le maʻi pe a pasi se tasi matua ia oe. E mafai foi ona atiaʻe lenei faaletonu o le kenera ma le leai o se talafaʻasolopito o aiga.

Faʻailoga autu o lenei faʻamaʻi o:

• O se ituaiga kanesa o le paʻu e taʻua o le basal cell carcinoma e tupu aʻe i le taimi o le talavou
• O le tuma e leai se kanesa o le auvae, ua taʻua ole kerotocystic odontogenic tumo e tupu foi i le taimi o le talavou

Isi faʻailoga aofia ai:

• Isu lautele
• Lugā gao
• Malulu, pupula o le muaulu
• Auvae e mau (i isi tulaga)
• Lautele-pupula mata
• Tuʻu i luga o alofilima ma alofilima

O le tulaga e ono aʻafia ai le neula ma faʻaoso atu ai i:

• Faʻafitauli tau mata
• Tutuli
• Le atoatoa le malosi o le mafaufau
• Faoa faamalosi
• Tumua o le faiʻai

O le tulaga e tau atu foi i ponaivi faaletonu, aofia ai:

• Fetivai o le tua (scoliosis)
• Paʻu ogaoga o le tua (kyphosis)
• Ivi asoaso le masani ai

Atonu ei ai se talaʻaga aiga o lenei faʻamaʻi ma se taimi ua tuanaʻi talaʻaga o basal cell paʻu kanesa.

E ono faʻaalia i suʻega:

• Tumua ole faiʻai
• Cysts i le auvae, lea e mafai ona taitai atu ai i le le masani ai atinaʻe nifo poʻo auvae gaui
• Faʻaletonu i le vaega lanu (iris) poʻo tioata o mata
• Fula ulu ona o le suavai i luga o le faiʻai (hydrocephalus)
• Faʻaletonu ivi asoaso

O suʻega e mafai ona faia e aofia ai:

• Echocardiogram o le fatu
• Suʻega ole genetic (i nisi tagata mamaʻi)
• MRI o le faiʻai
• Paʻu biopsy o tuma
• X-ray o ponaivi, nifo ma le ulu
• Ultrasound e siaki ai le faʻamaʻi neivi

E taua le suʻesuʻeina e le fomaʻi o le paʻu (dermatologist) i taimi uma, ina ia mafai ona togafitia kanesa o le paʻu a o laʻititi lava.

Tagata e iai lenei faʻamaʻi ono mafai foi ona vaʻaia ma togafitia e isi faʻapitoa, faʻamoemoeina i le itu o le tino ua aʻafia. Mo se faʻataʻitaʻiga, e mafai e se fomaʻi faʻapitoa i le kanesa (oncologist) ona togafitia ni patu i le tino, ma se fomaʻi tipitipi e mafai ona fesoasoani e togafitia faʻafitauli o ponaivi.

O le masani ona tulitatao ma le tele o fomaʻi faʻapitoa e taua mo le mauaina o se lelei iuga.

Tagata e maua i lenei tulaga e ono tupu:

• Tauaso
• Tumo o le faiʻai
• Tutuli
• Fractures
• Tumama ova
• Cardiac fibromas
• Paʻu faʻaleagaina ma matuitui maʻila ona o le paʻu kanesa

Faʻafesoʻotaʻi lau auaunaga faʻasoifua maloloina mo se taimi pe a fai:

• Oe poʻo ni tagata o le aiga e maua i le nevoid basal cell carcinoma syndrome, aemaise pe a fai o loʻo e fuafuaina e faia sau tama.
• E i ai sau tamaititi o loʻo maua i faʻamaʻi o lenei faʻamaʻi.

O ulugaliʻi ma se aiga talaʻaga o lenei faʻamaʻi atonu e mafaufau e faufautua i mea tau gafa a o leʻi maʻito.

O le le alu i fafo o le la ma le faʻaaogaina o le sunscreen e mafai ona fesoasoani e puipuia ai mai le basal cell i le paʻu o kanesa.

Aloʻese mai le paʻu e pei o x-ray. Tagata ma lenei tulaga e matua nofouta i le radiation. O le faʻaalia i le radiation e mafai ona oʻo atu ai i kanesa paʻu.

NBCC faʻamaʻi; Gorlin syndrome; Gorlin-Goltz syndrome; Basal cell nevus syndrome (BCNS); Basal cell kanesa - nevoid basal cell carcinoma faʻamaʻi

• Basal cell nevus syndrome - latalata i le alofilima
• Basal cell nevus syndrome - lua toto
• Basal cell nevus syndrome - foliga ma le lima
• Basal cell nevus syndrome
• Basal cell nevus syndrome - foliga

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Skelsey MK, Peck GL. Nevoid basal cell kanesa kanesa. I: Lebwohl MG, Heymann WR, Berth-Jones J, Coulson IH, eds. Togafitiga o le Paʻu Faamai: Auiliili togafitiga togafitiga. 5th ed. Filatelefia, PA: Elsevier; 2018: chap 170

Walsh MF, Cadoo K, Salo-Mullen EE, Dubard-Gault M, Stadler ZK, Offit K. genetic mea: tupuaga kanesa predisposition syndromes. I: Niederhuber JE, Armitage JO, Kastan MB, Doroshow JH, Tepper JE, eds. Abeloff's Clinical Oncology. 6th ed. Filatelefia, PA: Elsevier; 2020: chap 13.