Neoplasia endocrine badan (MEN) II

Noocyo badan oo loo yaqaan 'endocrine neoplasia', nooca II (MEN II) waa cilad ku dhacda qoysaska taas oo mid ama in ka badan oo qanjidhada 'endocrine' ah ay kacsadaan ama ay yeeshaan buro. Qanjidhada Endocrine ee inta badan ku lug leh waxaa ka mid ah:

• Qanjirka 'Adrenal' (qiyaastii waqtiga badhkiis)
• Qanjirka 'parathyroid' (20% waqtiga)
• Qanjirka 'thyroid' (ku dhowaad waqtiga oo dhan)

Multiple endocrine neoplasia (MEN I) waa xaalad la xiriirta.

Sababta Ragga II waa cillad ku timid hiddo-wadaha loo yaqaan RET. Ciladani waxay keentaa burooyin badan inay isla qof ka soo muuqdaan, laakiin daruuri maahan inay isla waqti isku mid ah soo baxaan.

Ku lug lahaanshaha qanjirka 'adrenal gland' badanaa waxaa leh buro loo yaqaan 'pheochromocytoma'.

Ku lug lahaanshaha qanjirka 'thyroid' badanaa waxaa leh buro loo yaqaan 'medullary carcinoma' oo ah qanjirka 'thyroid'.

Burooyinka ku jira qanjirka 'thyroid', 'adrenal', ama qanjidhada 'parathyroid' ayaa dhici kara sanado kala fog.

Cilladaan waxay ku dhici kartaa da 'kasta, waxayna si isku mid ah ugu dhacdaa ragga iyo dumarka. Cunsurka ugu weyn ee halista keena waa taariikh qoys RAGII II.

Waxaa jira labo nooc oo RAG II ah. Iyagu waa MEN IIa iyo IIb. MEN IIb wuu ku yaryahay.

Calaamaduhu way kala duwanaan karaan. Si kastaba ha noqotee, waxay la mid yihiin kuwa:

• Kareemada Medullary ee qanjirka 'thyroid'
• Pheochromocytoma
• Parathyroid adenoma
• Parathyroid hyperplasia

Si loo ogaado xaaladan, bixiyaha xanaanada caafimaadka wuxuu raadinayaa isbeddel ku dhaca hidda-wadaha RET. Tan waxaa lagu sameyn karaa baaritaanka dhiigga. Tijaabooyin dheeraad ah ayaa la sameeyaa si loo go'aamiyo hormoonnada soo-saarka badan.

Baadhitaanka jirka ayaa daaha ka qaadi kara:

• Qanjidhada qanjirada ee balaaran
• Qandho
• Dhiig kar
• Qiyaasta garaaca wadnaha
• Qanjira qanjidhada

Tijaabinta sawirka ee loo aqoonsaday burooyinka waxaa ka mid noqon kara:

• Sawirka CT-ga ee caloosha
• Sawirka kelyaha ama kaadi mareenka
• MIBG scintiscan
• MRI caloosha
• Baadhitaanka qanjirka 'thyroid'
• Ultrasound ee qanjirka 'thyroid'

Tijaabooyinka dhiigga waxaa loo isticmaalaa in lagu arko sida wanaagsan ee qanjirrada jirka qaarkood u shaqeeyaan. Waxay ka mid noqon karaan:

• Heerka Calcitonin
• Dhiigga alkaline phosphatase
• Kaalshiyam dhiig
• Heerka hormoonka parathyroid dhiig
• Fosfooraska dhiigga
• Katecholamines kaadi
• Kaadi kaadi metanephrine

Tijaabooyinka kale ama nidaamyada laga yaabo in la sameeyo waxaa ka mid ah:

• Cad ka-qaadista adrenal
• Qalabka korontada (ECG)
• Cad ka-qaadista qanjirka 'thyroid'

Qalliin ayaa loo baahan yahay si looga saaro cudurka 'pheochromocytoma', kaasoo nafta halis gelin kara sababo la xiriira hormoonnada uu sameeyo.

Marka loo eego kansarka 'medullary carcinoma' ee qanjirka 'thyroid', qanjirka 'thyroid' iyo qanjidhada ku xeeran waa in gebi ahaanba laga saaraa. Daaweynta beddelka hormoonka tayroodhka waxaa la bixiyaa qalitaanka ka dib.

Haddii cunugga lagu yaqaan inuu sido hiddo-wade hidde-side 'RET', qalliin looga saarayo qanjirka 'thyroid' ka hor inta uusan kansar noqon ayaa la tixgelinayaa Tan waa in lagala hadlaa takhtar aad u yaqaana xaaladan. Waxaa lagu sameyn lahaa da'da hore (ka hor da'da 5) dadka la yaqaan MEN IIa, iyo kahor da'da 6 bilood dadka qaba MEN IIb.

Pheochromocytoma badanaa ma aha kansar (benign). Cudurka 'Medullary carcinoma' ee tayroodhku waa kansar aad u dhib badan oo suuragal ah inuu dilo, laakiin ogaanshaha hore iyo qalliinka ayaa badanaa u horseedi kara daawo. Qalliinku ma daaweynayo Ragga II.

Faafitaanka unugyada kansarku waa dhibaato suurtagal ah.

Wac daryeel bixiyahaaga haddii aad dareento astaamaha cudurka MEN II ama haddii qof qoyskaaga ka mid ah uu helo baaritaanka noocaas ah.

Baadhitaanka qaraabada dhow ee dadka qaba MEN II waxay u horseedi kartaa ogaanshaha hore ee cilladda iyo kansarrada la xiriira. Tani waxay u oggolaan kartaa tallaabooyin looga hortagayo dhibaatooyinka.

Sipple syndrome; RAG II; Pheochromocytoma - RAGGA II; Kansarka qanjirka 'thyroid' - pheochromocytoma; Parathyroid cancer - cudurka pheochromocytoma

• Qanjirada 'Endocrine'

Shabakada Shabakada Shabakada Kansarka oo dhameystiran. Tilmaamaha tababarka caafimaadka ee cudurka kansarka (NCCN guideines): burooyinka neuroendocrine. Nooca 1.2019. www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. La cusbooneysiiyay Maarso 5, 2019. La helay Maarso 8, 2020.

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Nieman LK, Spiegel AM. Cilladaha polyglandular. Gudaha: Goldman L, Schafer AI, eds. Daawada Goldman-Cecil. 26aad. Philadelphia, PA: Elsevier; 2020: cutubka 218.

Tacon LJ, Learoyd DL, Robinson BG. Noocyo badan oo endocrine neoplasia ah 2 iyo medullary thyroid carcinoma. Gudaha: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Dadka waaweyn iyo carruurta. 7aad ed. Philadelphia, PA: Elsevier Saunders; 2016: cutubka 149.