Uninzi lwe-endocrine neoplasia (MEN) II

Uninzi lwe-endocrine neoplasia, uhlobo II (MEN II) sisifo esidluliswa kwiintsapho apho elinye okanye nangaphezulu kwamadlala endocrine asebenza kakhulu okanye enza isimila. Amadlala e-Endocrine ahlala echaphazeleka kubandakanya:

• I-adrenal gland (malunga nesiqingatha sexesha)
• Idlala leParathyroid (iipesenti ezingama-20 zexesha)
• Idlala lengqula (phantse lonke ixesha)

Uninzi lwe-endocrine neoplasia (MEN I) yimeko enxulumene noko.

Unobangela we-MEN II sisiphene kwimfuza ebizwa ngokuba yi-RET. Esi siphene senza ukuba amathumba amaninzi avele kumntu ofanayo, kodwa hayi ngexesha elinye.

Ukubandakanywa kwe-adrenal gland kuhlala kudla ngokuba nethumba elibizwa ngokuba yi-pheochromocytoma.

Ukubandakanywa kwe-thyroid gland kudla ngokuba ne-tumor ebizwa ngokuba yi-medullary carcinoma ye-thyroid.

Izidumbu kwi-thyroid, i-adrenal, okanye i-parathyroid gland ingenzeka ngokuhlukana kweminyaka.

Esi sifo sinokuvela nangaliphi na ixesha, kwaye sichaphazela amadoda nabafazi ngokulinganayo. Eyona nto iphambili emngciphekweni yimbali yosapho ye-MEN II.

Zimbini iintlobo zeMEN II. Ziyi-MEN IIa kunye ne-IIb. I-MEN IIb ayiqhelekanga.

Iimpawu ziyahluka. Nangona kunjalo, ziyafana nezo:

• I-medullary carcinoma ye-thyroid
• Pheochromocytoma
• I-adenoma yeParathyroid
• I-Parathyroid hyperplasia

Ukuchonga le meko, umboneleli wezempilo ujonga utshintsho kwi-RET gene. Oku kunokwenziwa kuvavanyo lwegazi. Uvavanyo olongezelelweyo lwenziwa ukumisela ukuba zeziphi iihomoni eziveliswa ngaphezulu.

Uvavanyo lomzimba lunokubonisa:

• Ukwandiswa kwee-lymph node entanyeni
• Ifiva
• Igazi elonyukayo
• Inqanaba lentliziyo elikhawulezayo
• Amaqhuqhuva e-thyroid

Ukulinganisa iimvavanyo ezisetyenziselwa ukufumanisa amathumba kunokubandakanya:

• Ukuskena kwe-CT esiswini
• Ukucinga ngezintso okanye ureters
• IMIBG iscisciscan
• I-MRI yesisu
• Ukuskena kwe-thyroid
• I-Ultrasound ye-thyroid

Uvavanyo lwegazi lusetyenziselwa ukubona ukuba isebenza njani na idlala ethile emzimbeni. Banokubandakanya:

• Inqanaba leCalcitonin
• Igazi lealkaline phosphatase
• I-calcium yegazi
• Inqanaba legazi le-parathyroid
• I-phosphorus yegazi
• Iicatecholamines zomchamo
• Umchamo we-metanephrin

Olunye uvavanyo okanye iinkqubo ezinokuthi zenziwe zibandakanya:

• I-adrenal biopsy
• I-Electrocardiogram (ECG)
• I-biopsy yegilo

Utyando luyafuneka ukususa i-pheochromocytoma, enokubusongela ubomi ngenxa yehomoni eyenzayo.

Kwi-medullary carcinoma ye-thyroid, i-thyroid gland kunye ne-lymph nodes ezijikelezayo kufuneka zisuswe ngokupheleleyo. Unyango lwehyproorm lonyango olunikwa emva kotyando.

Ukuba umntwana uyaziwa ukuba aphathe i-RET gene mutation, utyando lokususa i-thyroid ngaphambi kokuba ibe ngumhlaza kuthathelwa ingqalelo. Oku kufuneka kuxoxwe nogqirha oqhelene kakhulu nale meko. Iya kwenziwa esemncinci (ngaphambi kweminyaka emi-5) kubantu abaziwayo i-MEN IIa, nangaphambi kobudala beenyanga ezi-6 kubantu abane-MEN IIb.

I-Pheochromocytoma idla ngokungabi ngumhlaza (enobungozi). I-Medullary carcinoma ye-thyroid ngumhlaza onobukrakra kwaye onokubulala, kodwa ukuxilongwa kwangaphambili kunye nokuhlinzwa kunokukhokelela kunyango. Utyando alunyangi i-MEN II engaphantsi.

Ukusasazeka kweeseli ezinomhlaza yinto enokwenzeka.

Tsalela umnxeba umboneleli wakho ukuba ubona iimpawu ze-MEN II okanye ukuba umntu kusapho lwakho ufumanisa isifo.

Ukuvavanya izihlobo ezisondeleyo zabantu abane-MEN II kunokukhokelela ekufumaneni kwangoko isifo kunye nomhlaza onxulumene nawo. Oku kunokuvumela amanyathelo okuthintela iingxaki.

Isifo seSipple; AMADODA II; Pheochromocytoma-AMADODA II; Umhlaza wedlala lengqula - pheochromocytoma; Umhlaza Parathyroid - pheochromocytoma

• Amadlala endocrine

Iwebhusayithi yeNethiwekhi yeCancer epheleleyo yoMhlaza. Isikhokelo sokusebenza kwezonyango kwi-oncology (izikhokelo ze-NCCN): i-neuroendocrine tumors. Inguqulelo 1.2019. www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. Ukuhlaziywa ngo-Matshi 5, 2019. Ifikeleleke ngo-Matshi 8, 2020.

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UNieman LK, uSpiegel AM. Ukuphazamiseka kwePolyglandular. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 218.

UTacon LJ, uLearoyd DL, uRobinson BG. Uhlobo lwe-endocrine neoplasia yohlobo lwe-2 kunye ne-medullary thyroid carcinoma. Ku: Jameson JL, De Groot LJ, de Kretser DM, et al, ii-eds. Endocrinology: Abantu abadala kunye nabantwana. Umhla wesi-7. IPhiladelphia, PA: Elsevier Saunders; I-2016: isahluko 149.